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Mutations in KCNH2 (coding for hERG) cause long or short QT syndromes (LQTS or SQTS), predisposing to life-threatening arrhythmias. More than 1000 variations in hERG sequences have been described, most of them are to be characterized. The objective is to standardize and accelerate the entire process necessary to phenotype hERG variants. An in silico evaluation was also included to characterize the structural impact of the variants. We selected 12 variants from patients with LQTS, and 1 with SQTS. We optimized the protocol to efficiently introduce mutations in hERG cDNA despite GC-rich sequences, using the Gibson assembly strategy. A pH-sensitive fluorescent tag was fused to hERG for fast-track evaluation of hERG cell trafficking. An optimized patch-clamp protocol of 35 sec was developed to evaluate hERG channel activity in transfected cells. R software was used to speed up analyses. In the present work, we observed a good correlation between cell surface expression, assessed by the pH-sensitive tag, and current densities. Also, we showed that the new biophysical protocol allows a significant gain of time in recording ion channel properties and provides extensive information on WT and variant channel biophycical parameters, that can all be recapitulated in a single parameter defined herein as the repolarization power. The impact of the variants on channel structure were also reported where structural information was available. These three readouts (trafficking, repolarization power and structural impact) define 3 pathogenicity indexes that may help clinical diagnosis. Fast track characterization of KCNH2 genetic variants shows its relevance to discriminate mutants that affect hERG channel activity from variants with undetectable effects. This information is meant to fill a patient database, as a basis for personalized medicine. The next steps will be to further accelerate the process using an automated patch-clamp system. [ABSTRACT FROM AUTHOR]

Summary: We describe a case of acute chest syndrome associated with Bordetella bronchiseptica pneumonia in a child with sickle cell disease. B. bronchiseptica is recognized as an important pathogen of the respiratory tract for a large variety of animal species. This zoonotic agent has been frequently associated with chronic and recurrent infections. In humans, the bacterium acts as an opportunistic pathogen affecting mostly immunocompromised patients or those with preexisting respiratory diseases. This case and literature review provides an opportunity to discuss the risk factors, treatment, follow-up, and prevention of such zoonotic infections in the context of a lack of cross-protection of new pertussis vaccines. [ABSTRACT FROM AUTHOR]

Abstract: A 0.3m diameter trickle-bed reactor filled with porous alumina cylinders is used to study the influence of the catalyst loading method on liquid distribution. The study compares the performances of the dense and the sock loading methods in terms of radial liquid spreading. Measurements were performed using two wire mesh tomography devices, placed, respectively, 0.33 and 0.96m below the liquid distributor. The obtained results show that, in opposition to general agreement, sock packed beds seem to distribute liquid better than dense packed beds. This surprising result may be tentatively explained by a larger anisotropy of the dense loading. [Copyright &y& Elsevier]

Résumé Introduction En France, la consommation de substances illicites est croissante. Certaines substances peuvent être accidentellement avalées par un enfant. Nous rapportons un cas d’intoxication par de l’ecstasy, une amphétamine produite illégalement, responsable d’une symptomatologie neurologique atypique chez un nourrisson. Observation Ce nourrisson de 11 mois a présenté brutalement un état d’agitation avec révulsion oculaire et mydriase bilatérale aréactive. Toute cause cérébrale a été éliminée. La recherche de toxique a mis en évidence une intoxication aux amphétamines et MDMA (pour 3,4-méthylènedioxy-méthamphétamine). L’évolution a été favorable en 24 h. Conclusion Bien que rares, les intoxications pédiatriques par de l’ecstasy sont plus fréquentes ces dernières années du fait de sa consommation au sein des foyers. Elle expose les jeunes enfants à l’ingestion accidentelle d’un comprimé d’ecstasy. Summary Introduction In France, the use of illicit drugs is increasing and therefore accidental poisoning may occur in infants and children. We report on a case of ecstasy poisoning in an infant who presented with atypical neurological symptoms. Case report An 11-month-old infant suddenly developed agitation with eye rolling and unreactive bilateral mydriasis. All neurologic causes were excluded. The search for toxicants revealed an intoxication with an amphetamine and MDMA. Progression was favorable in 24 h. Conclusion Although rare, pediatric intoxications by ecstasy have become more common in recent years, due to its consumption within households, exposing young children and infants to accidental ingestion of a tablet of ecstasy. [ABSTRACT FROM AUTHOR]

A wire-mesh tomography device was used to study the liquid saturation at 78 points covering the cross-sectional area of a 0.30m diameter trickle bed reactor. Measurements in the pulsing flow near the transition were done for glass beads and alumina cylinders using air and water as fluids. Local liquid saturation measurements allow identification of flow regime and pulse frequency for each of the 78 points considered. To the best of the authors’ knowledge this is the first time that such local measurements are done for the complete sectional area of a trickle bed reactor. Flow rate conditions at which different flow regimes coexist at the same horizontal plane of the column were identified. A remarkable influence of the initial liquid distribution on the observed flow regime was also observed. [Copyright &y& Elsevier]

Abstract: Introduction: K+ channels allow the passive and selective transport of K+ ions through the membranes. They control K+ homeostasis, neuronal and muscular excitabilities, and neurotransmitter and hormone release. Exegesis: K+ channels are composed of pore-forming subunits associated with regulatory subunits. Many different K+ channels have been identified. Conclusion: This diversity is stressed by the growing number of genetic and acquired diseases associated with these channels. [Copyright &y& Elsevier]

This paper carries on the investigation of the non-unitary su(2)−1/2 WZW model. An essential tool in our first work on this topic was a free-field representation, based on a c=−2 ηξ ghost system, and a Lorentzian boson. It turns out that there are several ‘versions’ of the ηξ system, allowing different su(2)−1/2 theories. This is explored here in details. In more technical terms, we consider extensions (in the c=−2 language) from the small to the large algebra representation and, in a further step, to the full symplectic fermion theory. In each case, the results are expressed in terms of su(2)−1/2 representations. At the first new layer (large algebra), continuous representations appear which are interpreted in terms of relaxed modules. At the second step (symplectic formulation), we recover a logarithmic theory with its characteristic signature, the occurrence of indecomposable representations. To determine whether any of these three versions of the su(2)−1/2 WZW is well defined, one conventionally requires the construction of a modular invariant. This issue, however, is plagued with various difficulties, as we discuss. [Copyright &y& Elsevier]

The bosonic βγ ghost system has long been used in formal constructions of conformal field theory. It has become important in its own right in the last few years, as a building block of field theory approaches to disordered systems, and as a simple representative—due in part to its underlying su(2)−1/2 structure—of non-unitary conformal field theories. We provide in this paper the first complete, physical, analysis of this βγ system, and uncover a number of striking features. We show, in particular, that the spectrum involves an infinite number of fields with arbitrarily large negative dimensions. These fields have their origin in a twisted sector of the theory, and have a direct relationship with spectrally flowed representations in the underlying su(2)−1/2 theory. We discuss the spectral flow in the context of the operator algebra and fusion rules, and provide a re-interpretation of the modular invariant consistent with the spectrum. [Copyright &y& Elsevier]

This paper presents experimental measurements and theoretical predictions of the velocity gradient in a fixed-bed reactor with single phase liquid flow. The local measurements are carried out by means of microelectrodes embedded in the packing of the bed, whereas the corresponding predictions are based on the surface renewal model. The fluid elements age distributions, needed in the surface renewal model, were determined using the variable-interval time-averaging (VITA) method. The parameters of VITA method, i.e. local interval time-averaging length a and threshold level k, were optimally selected and are equal to 3TC (TC is the integral time deduced from auto-correlation functions) and 0.9, respectively. It was shown that the surface renewal model is appropriate for predictions at high liquid flow rates, corresponding to a flow that behaves like a turbulent flow. Moreover, it was shown that with only one velocity gradient signal measurement, both experimental and theoretical values are obtained for a specified flow rate. The average value of the signal provides the experimental measurement, whereas its fluctuating part yields the corresponding theoretical prediction. [Copyright &y& Elsevier]

hERG potassium channels are essential for normal cardiac electrical activity. Mutations in the hERG gene (KCNH2) cause long QT syndrome, a disorder that predisposes individuals to life-threatening arrhythmias. More than 1000 mutations in hERG sequence are described in databases and most of them remains to be characterized. The objective of the work is to determine the consequences of the variants identified in LQT2 patients on the expression and function of the hERG channel. An optimized sequence hERG fused to pHluorin tag was synthesized. By amplifying mutated overlapping fragments, and using the Gibson assembly strategy, we constructed hERG-pHluorin plasmids with missense variations. CHO cells were transfected by electroporation with the Maxcyte system or Fugene method, and studied after 40 hours. Confocal images and flow cytometry analysis were used to evaluate the percentage of transfected cells and to quantify the membrane channel expression, through pH sensitive pHluorin tag. Conventional or automated patch clamp were used to evaluate the hERG activity. In the present work, we constructed hERG plasmids carrying 48 variations identified in patients in France. Higher efficiency of transfection was observed with electroporation as compared to the Fugene method. The activity of hERG-pHluorin was validated in manual patch-clamp, the pHluorin tag does not interfere with channel activity. Characterization of several mutations in conventional patch clamp showed difference in current amplitude. Automated patch-clamp data was compared to manual patch-clamp, as an initial validation for high throughput screening of all constructed mutations. High-throughput characterization of KCNH2 genetic variants is relevant to discriminate mutants that affect hERG channel activity from variants with undetectable effects. This information will be indicated in a patient database, as a basis of personalized medicine. [ABSTRACT FROM AUTHOR]

Objectifs: Définir les facteurs pronostiques de survie chez les nouveau-nés atteints de chylothorax congénital. Patients et Méthodes: Étude rétrospective mono-centrique des 29 nouveau-nés hospitalisés en réanimation pour chylothorax congénital entre 1999 et 2009. Recherche de facteurs de risque de décès en uni-varié par test de Fisher ou de Mann-Whitney. Résultats: 11/29 enfants sont décédés (38 %), 6 d’insuffisance respiratoire, 3 d’insuffisance rénale organique et 2 de cause mixte. Sur 4 autopsies, 2 ont retrouvé une hypoplasie pulmonaire, 1 des lymphangiectasies pulmonaires. Les facteurs de risque de décès sont : l’importance de la prématurité (p = 0,039), l’absence de drainage anténatal (64 % de décès versus 22 % ; p = 0,048), la présence d’une anasarque postnatale (75 % de décès versus 24 % ; p = 0,028), la naissance en état de mort apparente (p = 0,01), la nécessité d’une évacuation pleurale postnatale (p = 0,019), l’association à une pathologie générale (83 % de décès versus 26 % ; p = 0,018). Conclusions: La sévérité du chylothorax congénital est principalement liée à son association avec une insuffisance respiratoire constitutionnelle, persistant malgré un bon contrôle de l’épanchement pleural postnatal. [ABSTRACT FROM AUTHOR]

The aim of this study was to describe severe forms of novel coronavirus disease 2019 in children, including patient characteristics, clinical, laboratory, and imaging findings, as well as the disease management and outcomes. This was a retrospective, single-center, observational study conducted in a pediatric intensive and high-dependency care unit (PICU, HDU) in an urban hospital in Paris. All patients, aged from 1 month to 18 years, admitted for confirmed or highly suspected SARS-CoV-2 were included. We analyzed the data of 27 children. Comorbidities (n = 19, 70%) were mainly neurological (n = 7), respiratory, (n = 4), or sickle cell disease (n = 4). SARS-CoV-2 PCR results were positive in 24 children (nasopharyngeal swabs). The three remaining children had a chest CT scan consistent with COVID-19. Respiratory involvement was observed in 24 patients (89%). Supportive treatments were invasive mechanical ventilation (n = 9), catecholamine (n = 4), erythropheresis (n = 4), renal replacement therapy (n = 1), and extracorporeal membrane oxygenation (n = 1). Five children died, of whom three were without past medical history. This study highlighted the large spectrum of clinical presentation and time course of disease progression as well as the non-negligible occurrence of pediatric life-threatening and fatal cases of COVID-19 mostly in patients with comorbidities. Additional laboratory investigations are needed to further analyze the mechanism underlying the variability of SARS-Cov-2 pathogenicity in children. [ABSTRACT FROM AUTHOR]

Neurological involvement is frequent in inherited metabolic disease of the intoxication type. Hyperammonemic coma related to these diseases may cause severe neurological sequelae. Early optimal treatment is mandatory combining metabolite scavengers (MS) and sometimes continuous veno-venous hemodialysis (CVVHD). We aimed to describe the therapeutic management of hyperammonemia in neonates upon diagnosis of their metabolic disease and to compare neonates managed with MS alone or with both MS and CVVHD. We conducted a retrospective study including all neonates admitted for initial hyperammonemia to the pediatric intensive care unit of a Reference Center of Inherited Metabolic Diseases, between 2001 and 2012. The study included 35 neonates. Before admission, MS were initiated for 11 neonates. At admission, the median ammonia levels were 391 μmol/L and were significantly lower in neonates who received MS before admission. At admission, ammonia levels were 644 μmol/L in dialyzed and 283 μmol/L in non-dialyzed neonates. The median time to reach a 50% decrease of the initial ammonia levels was significantly shorter in dialyzed neonates; however, the normalization of ammonia levels was similar between dialyzed and non-dialyzed neonates. Hemodynamic disorders were more frequent in dialyzed neonates. MS represent an effective treatment for hyperammonemia and should be available in all pediatric units to avoid the need for CVVHD. Although CVVHD enhances the kinetics of toxic metabolite decrease, it is associated with adverse hemodynamic effects. [ABSTRACT FROM AUTHOR]

Aim Identifying early clinical and biological factors associated with severe forms of postdiarrheal hemolytic uremic syndrome (D + HUS) that may help practitioners determine appropriate treatment. Methods This retrospective study was conducted in 49 children with D + HUS between 2001 and 2011. Severe forms were defined as occurrence of one of the following conditions: death, major neurological involvement, cardiovascular involvement, and/or the presence of sequelae (neurological, cardiovascular, pancreatic, or renal). Results During the acute phase, 35 children exhibited at least one type of extrarenal involvement including 13 severe forms with a median delayed occurrence after admission of 4.5 days (range: 1–8) for comatose children and 5 days (range: 2–6) for cardiovascular involvement; 32 children required dialysis and three died. In multivariate analysis, (i) major neurological involvement ( n = 13), (ii) dialysis ( n = 32), and (iii) sequelae ( n = 12) were associated with (i) fever during the prodromal phase requiring dialysis at admission, (ii) C-reactive protein level (CRP) > 22 mg/L at admission, and (iii) major neurological involvement and a white blood cell count (WBC) > 20 × 10 3 /mm 3 during the acute stage, respectively. Conclusions D + HUS is a multiorgan disease with a delayed occurrence of life-threatening extrarenal organ involvement. Severe forms appear to be associated with early biological and clinical inflammatory parameters. [ABSTRACT FROM AUTHOR]

Résumé L’œdème aigu hémorragique du nourrisson (OAHN) est une vascularite rare et bénigne survenant chez des nourrissons de 4 à 24 mois. Les lésions cutanées, prenant parfois l’aspect d’un purpura ecchymotique ou en nappe, peuvent faire évoquer le diagnostic de purpura fulminans lorsqu’une fièvre y est associée et entraîner alors la mise en route d’une antibiothérapie. Nous rapportons le cas d’un nourrisson de 7 mois dont la présentation clinique initiale d’OAHN a fait suspecter un purpura fulminans. L’évolution rapide des lésions a fait instaurer une antibiothérapie par céfotaxime et amikacine. Le diagnostic d’OAHN a été porté le lendemain devant l’aspect des lésions cutanées, la stabilité hémodynamique de l’enfant et la négativité du bilan bactériologique. Un entérovirus (Coxsackie virus B5) a été mis en évidence secondairement par réaction de polymérisation en chaîne spécifique (PCR) dans le liquide céphalorachidien. Après arrêt de l’antibiothérapie, l’évolution a été spontanément favorable. L’imputabilité de l’entérovirus dans le déclenchement de cet OAHN est discutée. Summary Acute hemorrhagic edema of infancy is a rare but benign vasculitis occurring in infants aged from 4 to 24 months. Skin lesions can take various forms, including extensive hemorrhagic purpura, and can therefore be mistaken for purpura fulminans if associated with fever, which leads to initiating broad-spectrum antibiotic treatment. In the present case, we describe a 7-month-old boy with acute hemorrhagic edema of infancy and rapidly extensive purpura lesions that led to intravenous cefotaxime and amikacin treatment. Diagnosis was made on the next day by a dermatologist, based on the typical aspect of skin lesions, hemodynamic stability, and negative bacteriological samples. Coxsackie virus B5, a pathogenic enterovirus, was found by specific PCR in cerebrospinal fluid. The outcome was spontaneously favorable after discontinuation of antibiotics on day 2. We discuss the imputability of the enterovirus in triggering this case of acute hemorrhagic edema of infancy. [ABSTRACT FROM AUTHOR]

Optical imaging of retinal intrinsic signals is a relatively new method that provides spatiotemporal patterns of retinal activity through activity-dependent changes in light reflectance of the retina. The exact physiological mechanisms at the origin of retinal intrinsic signals are poorly understood and there are significant inter-species differences in their characteristics and cellular origins. In this study, we re-examined this issue through pharmacological dissection of retinal intrinsic signals in the rabbit with simultaneous ERG recordings. Retinal intrinsic signals faithfully reflected retinal activity as their amplitude was strongly associated with stimulation intensity (r2=0.85). Further, a strong linear relation was found using linear regression (r2=0.98) between retinal intrinsic signal amplitude and the ERG b wave, which suggests common cellular origins. Intravitreal injections of pharmacological agents were performed to isolate the activity of the retina's major cell types. Retinal intrinsic signals were abolished when the photoreceptors' activity was isolated with aspartate, indicative that they are not at the origin of this signal. A small but significant decrease in intrinsic response (20%) was observed when ganglion and amacrine cells' activity was inhibited by TTX injections. The remaining intrinsic responses were abolished in a dose-dependent manner through the inhibition of ON-bipolar cells by APB. Our results indicate that, in rabbits, retinal intrinsic signals reflect stimulation intensity and originate from the inner retina with a major contribution of bipolar cells and a minor one from ganglion or amacrine cells. [ABSTRACT FROM AUTHOR]

Neuroligin 1 (NLGN1) is a postsynaptic adhesion molecule that determines N-methyl- d -aspartate receptor (NMDAR) function and cellular localization. Our recent work showed that Nlgn1 knockout (KO) mice cannot sustain neuronal activity occurring during wakefulness for a prolonged period of time. Since NMDAR-dependent neuronal activity drives an important vascular response, we used multispectral optical imaging to determine if the hemodynamic response to neuronal stimulation is modified in Nlgn1 KO mice. We observed that Nlgn1 KO mice show a 10% lower response rate to forepaw electrical stimulation compared to wild-type (WT) and heterozygote (HET) littermates on both the contra- and ipsilateral sides of the somatosensory cortex. Moreover, Nlgn1 mutant mice showed an earlier oxyhemoglobin peak response that tended to return to baseline faster than in WT mice. Analysis of the time course of the hemodynamic response also showed that HET mice express a faster dynamics of cerebrovascular response in comparison to WT. Taken together, these data are indicative of an altered immediate response of the brain to peripheral stimulation in Nlgn1 KO mice, and suggest a role for NLGN1 in the regulation of cerebrovascular responses. [ABSTRACT FROM AUTHOR]

Résumé: Introduction et objectifs: Préciser la connaissance des soignants de réanimation pédiatrique (RP) sur la loi Léonetti, connaître leurs opinions vis-à-vis des décisions de limitation ou arrêt des thérapeutiques (LAT) et leur ressenti sur la façon dont ces décisions ont été prises et appliquées. Matériel et méthode: Étude multicentrique par questionnaire durant le dernier trimestre 2010. Résultats: Mille cinq personnels paramédicaux et 334 médecins ont répondu. Plus de 85 % avaient une bonne connaissance des grands principes de la Loi. Plus de 80 % des soignants adhéraient aux LAT lorsqu’elles concernaient une suppléance respiratoire, hémodynamique ou rénale. En revanche, l’arrêt de la nutrition et de l’hydratation artificielle était récusé ou générait des réticences chez la majorité des répondants. Alors que la délibération collégiale était estimée indispensable par l’ensemble des soignants, les personnels paramédicaux trouvaient beaucoup plus souvent que les médecins que leur implication réelle était insuffisante. La qualité des fins de vie était jugée très favorablement par les soignants. Cependant, les réponses concernant les modalités d’application des LAT suggèrent des interprétations très différentes de la Loi : certains respectaient les principes des soins palliatifs tels qu’ils sont définis par le code de santé publique, alors que 40 % des médecins et 64 % des soignants jugeaient ű acceptable Ƈ de provoquer un décès si la décision était collégiale. Conclusion: Cette étude est la première à montrer que les soignants des unités de RP françaises ont une bonne connaissance de la loi Léonetti. Il persiste cependant des divergences importantes sur les pratiques jugées légalement acceptables en fin de vie. [ABSTRACT FROM AUTHOR]

L’infection à Parvovirus B19 (PB19) est une maladie infantile bénigne et fréquente. De rares cas de myocardites fatales ont été rapportés, dont la physiopathologie demeure incertaine. Nous rapportons deux nouveaux cas de myocardites fulminantes à PB 19 : 2 enfants sains qui, quelques jours suivant l’apparition de symptômes évoquant une infection banale à PB19, sont brutalement décédés d’incompétence myocardique. Le virus a été isolé dans le sérum des 2 patients par PCR ; les sérologies confirmaient l’infection récente à PB 19. L’analyse histologique post-mortem complète du tissu cardiaque a permis le diagnostic de myocardite fulminante en montrant un infiltrat inflammatoire majeur. Ces constatations sont en accord avec les hypothèses actuelles qui écartent un rôle cytopathogène du virus et privilégient une atteinte indirecte du myocarde. La myocardite aigue grave à PB19 de l’enfant, probablement de nature inflammatoire ou post-infectieuse, serait due à une dysrégulation de la réponse immune de l’hôte : elle serait disproportionnée et responsable de lésions myocardiques massives. Aucun critère clinique ne peut malheureusement à l’heure actuelle prédire l’évolution bien que très rarement dramatique de cette infection banale. [ABSTRACT FROM AUTHOR]

Summary: Introduction: Although rare, salicylate intoxication through the skin should not be ignored as it can be severely life-threatening. We describe an original case of accidental poisoning with salicylates topically applied to the scalp of a 6-week-old infant. Clinical report: A 6-week-old infant, with no prior history, was admitted to the pediatric intensive care unit for treatment of severe disorders of consciousness associated with significant tachypnea. Laboratory results revealed metabolic acidosis with elevated anion gap, ketonuria, and normal glycemia. Initial assessment ruled out the hypothesis of accidental ingestion of salicylates. However, the presence of salicylic acid derivatives in organic acid chromatography, confirmed by plasma salicylate levels at 580mg/L, ultimately re-established the diagnosis. Further inquiry retrospectively highlighted the prolonged topical application in occlusion (3 days) of an extemporaneous preparation containing 23% salicylic acid on the scalp. The course after urine alkalinization was rapidly favorable without sequelae. Comment and conclusions: Salicylate intoxication is potentially lethal, particularly in infants under 12 months of age. The vast majority of these intoxications result from accidental ingestion. The present observation underscores the original and undescribed risk of intoxication due to a localized application to the scalp. In the presence of warning symptoms, salicylate poisoning should be investigated, including topical application of salicylic acid, even if localized. Careful attention should be paid to following the indications of use of this product in terms of concentration, characteristics of the infant, and exposed skin. The use of extended topical application of salicylic acid in concentrations greater than 3% should be avoided. [Copyright &y& Elsevier]

Abstract: We evaluated 131 patients (6 months–14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n =12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx. [Copyright &y& Elsevier]