RESUMEN
OBJECTIVES: European Crohn's Colitis Organization (ECCO) and the European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines recommend the early use of anti-tumor necrosis factor (TNF) biologicals in pediatric Crohn disease (CD) patients with positive predictors for poor outcome. The objective of the present study was to compare early "Top-Down" use of adalimumab (ADA) immunomodulator/biologics-naive patients to conventional "Step-Up" management. METHODS: One hundred and twenty consecutive patients with a confirmed diagnosis of CD and treated with ADA between 2008 and 2019 were included and allocated to the ADA-Top Down (n = 59) or ADA-Step Up group (n = 61). The primary endpoint was prolonged steroid-/enteral nutrition-free clinical remission at 24 months, defined by a weighted Pediatric Crohn's Disease Activity Index (wPCDAI) < 12.5. Clinical and biological data were collected at 12 and 24 months. RESULTS: At start of ADA, disease activity was comparable between the ADA-Top Down group and the ADA-Step Up group (wPCDAI = 31 ± 16 vs 31.3 ± 15.2, respectively, P = 0.84). At 24 months, the remission rate was significantly higher in the ADA-Top Down group (73% vs 51%, P < 0.01). After propensity score, the Top-Down strategy is still more effective than the Step-Up strategy in maintaining remission at 24 months [hazard ratio (HR) = 0.36, 95% CI (0.15-0.87), P = 0.02]. Patients in the ADA-Top Down group were mainly on monotherapy compared to patients in the ADA-Step Up group (53/55 vs 28/55 respectively, P < 0.001). Serum levels of ADA were higher in the ADA-Top Down group than in the ADA-Step Up group (12.8 ± 4.3 vs 10.4 ± 3.9 µg/mL, respectively, P < 0.01). There were no serious adverse events. CONCLUSIONS: Early use of ADA appears to be more effective in maintaining relapse-free remission at 2 years, while using it as monotherapy. These findings further favor the recommendation of early anti-TNF use in high-risk CD patients.
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Adalimumab , Enfermedad de Crohn , Niño , Humanos , Adalimumab/uso terapéutico , Enfermedad de Crohn/tratamiento farmacológico , Estudios de Seguimiento , Infliximab/uso terapéutico , Inducción de Remisión , Resultado del Tratamiento , Inhibidores del Factor de Necrosis Tumoral/uso terapéuticoRESUMEN
Effusions can show some surprises. We document the case of a fourteen-month-old male patient with short-bowel syndrome, hospitalized in a cardiology unit, receiving a chronic parenteral nutrition by a Broviac® catheter. The patient presented several thrombosis following iterative catheter replacements. In parallel with superior vena cava plasty, a right intra-atrial Broviac® catheter was placed in the absence of other peripheral venous accesses. This device has a cutaneous exit site to allow for infusion of a hyperosmolar lipid emulsion. Seven days later, a milky liquid was secreted from pericardial/mediastinal redon. A gel lipoprotein electrophoresis of the fluid suggested a preliminary diagnosis of chylopericardium. However, biochemical testing of certain analytes evoked a parenteral nutrition-related pericardial effusion and a possible pseudochyloperitoneum caused by the shearing of a migrated Broviac® in pericardium. The patient, on a fat-free diet, was admitted to the ICU to drain the effusion and reposition the catheter, with success. In the light of new datas on the interference of parenteral lipid emulsions with the lipoproteins gel electrophoresis, we will try to determine whether the apparent presence of chylomicrons in the gel would be the sign of a lesion of the lymphatic system, or rather the result of a contamination by artificial chylomicron in the lipid emulsion, if not the sign of contaminated blood. In our article, we highlight several considerations in identifying and confirming cases of pericardial effusion, such as chylopericardium and parenteral nutrition-related one, as well as points concerning the use of lipid emulsions for pediatric patients with short-bowel syndrome.
Les liquides d'épanchements peuvent renfermer quelques surprises. Nous documentons le cas d'un patient de quatorze mois, hospitalisé en cardiologie, présentant un syndrome de grêle court et recevant une nutrition parentérale au long cours par cathéter Broviac®. Le patient présentait de multiples occlusions veineuses consécutives aux changements itératifs du dispositif. En parallèle d'une plastie de la veine cave supérieure, un Broviac® a été posé en intra-atrial droit devant l'absence d'autres abords veineux périphériques. Ce dispositif comporte un orifice de sortie sous-cutané pour apporter une solution de nutrition hyperosmolaire de type émulsion lipidique. Le liquide recueilli dans les drains péricardiques en post-opératoire est lactescent, particulièrement à partir du septième jour. Le lipidogramme du liquide d'épanchement péricardique semble conclure à la présence de chylomicrons - un chylopéricarde. Cependant, le dosage de certains analytes penche en faveur d'un perfusopéricarde, probablement pseudochyleux, lié au cisaillement du Broviac® dont l'extrémité a migré de l'oreillette droite au péricarde. Le patient, sous régime sans graisses, sans nutrition parentérale, sera réopéré pour drainer l'épanchement et repositionner le cathéter, avec succès. À la lumière de données originales quant à l'interférence des émulsions lipidiques sur le lipidogramme, nous tâcherons de déterminer si l'apparente présence de chylomicrons sur le gel serait le témoin d'une réelle lésion du lymphatique, ou plutôt le fruit d'une contamination par l'émulsion, si ce n'est par le sang. Des considérations au sujet des épanchements péricardiques, dont les chylopéricarde et nutripéricarde, ainsi que sur les émulsions lipidiques pédiatriques dans le contexte du grêle court émailleront ce travail.
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Derrame Pericárdico , Síndrome del Intestino Corto , Humanos , Masculino , Niño , Lactante , Derrame Pericárdico/diagnóstico , Derrame Pericárdico/etiología , Emulsiones , Vena Cava Superior , Nutrición Parenteral , LípidosRESUMEN
OBJECTIVES: The first ECCO-ESPGHAN guidelines for the medical management of pediatric Crohn disease (CD) were published in 2014. Whether their implementation, and the consequent increased use of an upfront anti-tumor necrosis factor therapy, have changed the course of the disease has not been investigated yet. We aimed at comparing the evolution of pediatric CD patients diagnosed and treated before and after 2014. METHODS: Single-center retrospective study including all children diagnosed with CD from January 2010 to December 2018. Patients diagnosed between 2010 and 2014 (group 1) were compared to those diagnosed after 2014 (group 2). For each patient, at baseline and every 6-month, number of relapses, the occurrence of complication, therapy received and biological parameters were noted, as well as any endoscopic or radiologic evaluation. RESULTS: One hundred and fifty-four patients were included in the analysis, 78 (51%) diagnosed after 2014. The cumulative probability of a relapse-free and surgery-free course was significantly higher for patients treated according to the guidelines (log rank hazard ratio [HR] = 1,818, P = 0.003 and HR = 3,15, 95% confidence interval, P = 0.04, respectively). Mucosal healing rate was significantly higher among patients of group 2 at 1 and 2âyears (P = 0.04 and P = 0.05, respectively), while no significant difference was observed for transmural healing rates, as well as for the risk of complications. CONCLUSIONS: The implementation of the 2014 CD guidelines appears to have a significant impact on disease outcomes, with a significantly lower risk for relapse and surgery, while no effect could be observed on the risk of developing complications.
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Enfermedad de Crohn , Niño , Enfermedad de Crohn/diagnóstico , Humanos , Infliximab/uso terapéutico , Recurrencia , Estudios Retrospectivos , Factor de Necrosis Tumoral alfaRESUMEN
BACKGROUND: Composite lipid emulsions containing soybean oil (30%), medium-chain triglycerides (30%), olive oil (25%), and fish oil (15%) (SMOF) are now widely used. OBJECTIVES: We aimed to evaluate the tolerance, the efficiency, and the erythrocyte fatty acid (FA) profile for children on long-term home parenteral nutrition (HPN) receiving a composite fish oil-based emulsion (FOLE). METHODS: At baseline, children (n = 46) with severe intestinal failure highly dependent on parenteral nutrition (PN) for ≥1 y were included in the study when they had received the composite FOLE for >6 mo. Out of this baseline group, only 25 children remained highly PN-dependent (SMOF1, n = 25) and could be assessed a second time, 2.4 y later (SMOF2, n = 25). An independent control group ("weaned off PN" group; n = 24) included children who had been weaned off PN for >2 y (median: 4 y). RBC-FA composition was established by GC-MS. Growth parameters, plasma citrulline, conjugated bilirubin, FA profiles, and the Holman ratio (20:3ω-9/20:4ω-6) were compared between groups. RESULTS: No difference for growth parameters, citrulline, and bilirubin was observed between the SMOF groups after 2.4 y (0.2 < P < 0.8). The weaned-off group did not differ from the SMOF groups for growth parameters (0.2 < P < 0.4) but citrulline was higher (P < 0.0001) and conjugated bilirubin lower (P < 0.01). The composite FOLE induced higher proportions of EPA (20:5n-3) (8.4% ± 2.9%) and DHA (22:6n-3) (11.7% ± 2.2%) than what was observed in weaned-off children (0.8% ± 0.4% and 6.6% ± 2.3%, respectively) but lower proportions of arachidonic acid (20:4n-6). However, the Holman ratio did not vary between groups (P = 0.9), whereas the PUFA concentrations varied widely. CONCLUSIONS: Long-term use of the composite FOLE was well tolerated in HPN-dependent children. The RBC-FA profile alterations were consistent with the ω-3 PUFA-enriched composition of this emulsion without evidence of essential FA deficiency.
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Membrana Eritrocítica/química , Ácidos Grasos Omega-3/administración & dosificación , Ácidos Grasos/sangre , Insuficiencia Intestinal/sangre , Nutrición Parenteral en el Domicilio/métodos , Bilirrubina/sangre , Niño , Preescolar , Estudios Transversales , Emulsiones Grasas Intravenosas , Femenino , Aceites de Pescado/administración & dosificación , Alimentos Fortificados , Humanos , Insuficiencia Intestinal/terapia , Masculino , Aceite de Oliva/administración & dosificación , Aceite de Soja/administración & dosificación , Resultado del Tratamiento , Triglicéridos/administración & dosificaciónRESUMEN
BACKGROUND AND AIMS: Home Parenteral Nutrition (HPN) is the cornerstone management for children suffering from chronic intestinal failure (CIF). In France, HPN is organized from a network of 7 certified centers located in University Hospitals spread across the national territory. This study aims to review the data involving children on HPN over a 6-years period in France to outline the global and continuous improvement in care. PATIENTS AND METHODS: This cross-sectional study included all children enrolled in any of the 7 French HPN certified centers from January 1st, 2014 to December 31st, 2019. Data was recorded from annual databases provided by each center regarding: age at inclusion, indication and duration of HPN, type of intravenous lipid emulsion (ILE), outcome [PN weaning off, transfer to adult center, death, intestinal transplantation (ITx)], rate of catheter-related bloodstream infections (CRSBIs) for 1000 days of HPN, Taurolidine lock procedure (TLP) use and prevalence of cholestasis defined as conjugated bilirubin ≥20 µmol/l. RESULTS: The number of patients increased by 43.6% from 268 in 2014 to 385 in 2019. According to the year of follow up, the indications for HPN were short bowel syndrome (SBS) (42.3-46.6%), congenital enteropathies (CE) (18.5-22.8%), chronic intestinal pseudo-obstruction syndrome (CIPOS) (13.0-16.3%), long segment Hirschsprung's disease (LSHD) (9.7-13.3%), Crohn's disease (CD) (1.6-2.6%) and other non-primary digestive diseases (NPDD) such as immune deficiency, cancer or metabolic disease (4.0-9.2%). The median age at discharge on HPN decreased from 11.7 months in 2014 to 8.3 months in 2019 (p < .001). By December 31st, 2019, 44.8% of children had left the HPN program after a median duration ranging between 39.9 and 66.4 months. Among these patients, 192 (74.2%) were weaned off PN (94.7% SBS), 41 (15.8%) were transferred to adult centers for CIPOS (42%), SBS (31%) or CE (27%), 21 died (8.1%) - mostly in relation to cancer or immune deficiency - and 5 were transplanted (1.9%): 4 underwent combined liver-intestine transplantation for LSHD (n = 2), SBS, CE and one multivisceral Tx for CIPOS. The use of a composite fish-oil based ILE increased from 67.4% in 2014 to 88.3% in 2019 (p < 0.001). CRBSIs dropped from 1.04 CRSBIs per 1000 days HPN in 2014 to 0.61 in 2019 (p < 0.001) while meantime, the percentage of children receiving TLP increased from 29.4% to 63.0% (p < 0.001). The prevalence of cholestasis (conjugated bilirubin ≥ 20 µmol/l) was low and stable between 4.1 and 5.9% of children during the study period. CONCLUSION: In France, the number of children enrolled in a HPN program continuously increased over a 6 years period. SBS is the leading cause of CIF requiring HPN. The rate of CRBSIs dropped dramatically as the use of TLP increased. Mortality rate was low and mainly in relation to the underlying disease (cancer, immune deficiency). Cholestasis and intestinal Tx remained very rare.
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Enfermedades Intestinales/terapia , Insuficiencia Intestinal/terapia , Nutrición Parenteral en el Domicilio/estadística & datos numéricos , Nutrición Parenteral en el Domicilio/tendencias , Adolescente , Niño , Preescolar , Estudios Transversales , Manejo de la Enfermedad , Francia/epidemiología , Servicios de Atención de Salud a Domicilio/organización & administración , Humanos , Lactante , Mejoramiento de la CalidadRESUMEN
BACKGROUND & AIMS: Long-term parenteral nutrition (PN) is the mainstay of the therapeutic strategy in intestinal failure (IF) due to neonatal short bowel syndrome (SBS). Our aim was to identify prognostic factors for PN weaning and to assess if measuring plasma citrulline concentrations over time could account for the intestinal adaptation in progress. METHODS: This retrospective study included children with neonatal SBS with surgical measurement of the residual bowel length and repeated plasma citrulline assessments during a 4-year follow-up. The degree of IF was assessed by the PN dependency index (PN caloric intake/Resting energy expenditure). The analysis was carried out according to SBS anatomical groups: end-jejunostomy (type 1), jejuno-colic (type 2) and jejuno-ileal anastomosis (type 3). RESULTS: Fifty-five patients (8 type 1, 27 type 2, 20 type 3) were included. None of the patients with SBS type 1, 11 (41%) with type 2 and 11 (55%) with type 3 were weaned off during the follow-up period. Plasma citrulline levels significantly increased with time in patients who were finally weaned off PN; conversely, the levels did not consistently increase in patients who were still on PN at the end of the study period. There was an inverse relationship between plasma citrulline levels and the PN dependency index. The increasing citrulline levels had a positive effect on the probability of weaning, 2.7 times higher for each point increase in citrulline. No significant effect of age and residual bowel length at baseline was found. CONCLUSION: The increased plasma citrulline level over time in addition to the SBS anatomical type is a reliable marker for subsequent PN weaning. The prediction of PN weaning assessed solely by the residual bowel length or a single measurement of citrulline is insufficient and should also take into account the anatomical type of SBS and repeated measurements of plasma citrulline levels.
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Citrulina/sangre , Insuficiencia Intestinal/sangre , Nutrición Parenteral , Síndrome del Intestino Corto/sangre , Adaptación Fisiológica , Metabolismo Basal , Biomarcadores/sangre , Preescolar , Ingestión de Energía , Enterostomía/métodos , Enterostomía/estadística & datos numéricos , Femenino , Humanos , Lactante , Recién Nacido , Insuficiencia Intestinal/etiología , Insuficiencia Intestinal/terapia , Masculino , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Síndrome del Intestino Corto/complicaciones , Síndrome del Intestino Corto/terapia , Factores de Tiempo , Resultado del Tratamiento , DesteteRESUMEN
BACKGROUND: Fecal incontinence (FI) secondary to chronic retentive constipation is a frequent demand in pediatric gastroenterology clinics. The management of constipation in children includes laxatives (polyethylene glycol, PEG), enhanced toilet training, and dietary advice. Biofeedback is a possible treatment for children above the age of 7â¯years with resistant FI. AIM: To analyze any changes in volume to trigger defecation (VTD) and envy score over the course of biofeedback sessions according to clinical response. METHODS: In this retrospective study, we reviewed the medical records of 23 children diagnosed with FI according to the Rome IV criteria and treated with biofeedback. For each biofeedback session, a mean VTD by subject was measured. At the end, therapy was considered a success if soiling disappeared and a failure if any persisted. The need to defecate expressed by the child was described as an envy score. A 0-10 visual analog scale was used to express the intensity of this sensation. Follow-up involved calling the parents 12 months after the biofeedback sessions had ended to assess symptoms remotely. RESULTS: The study included 19 boys and 4â¯girls with a median age of 10 years. Patients' ages ranged between 7 and 17â¯years. None of them had any associated neurological disorders. All children had FI for >1â¯year. The median number of soiling episodes per week was 7. The average number of biofeedback sessions was 3 (range 1-5). At the end of the rehabilitation sessions, 12 children (52%) were in the "success" group. In the latter, median VTD decreased from 97â¯ml to 70â¯ml between the first and last session. In the "failure" group, VTD decreased from 120â¯ml to 100â¯ml. The between-group difference in the median VTD at the first session was not statistically significant. The last observation carried forward (LOCF) VTD was significantly lower in the "success" group compared to the "failure" group (70â¯ml versus 100â¯ml, pâ¯=â¯0.03). Median envy scores decreased during the biofeedback sessions with no statistical difference between the groups at the last session. Follow-up of children in the "success" group one year after the last biofeedback session revealed that 10 patients had no relapse (83%) and 2 were lost to follow-up. CONCLUSIONS: Biofeedback might be an effective tool for the management of FI resistant to medical treatment in children.
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Incontinencia Fecal , Adolescente , Biorretroalimentación Psicológica , Niño , Estreñimiento/terapia , Incontinencia Fecal/terapia , Femenino , Humanos , Masculino , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND & AIMS: Children on long-term home parenteral nutrition (HPN) are at increased risk of suboptimal growth and metabolic bone disease (MBD) i.e. decreased bone mineral density (BMD). The aims of this cross-sectional study were to assess growth and bone health in children on long term HPN and to identify risk factors for MBD. METHODS: Children above the age of 5 years, stable on HPN for more than 2 years were included. Medical files were reviewed retrospectively and included demographics, gestational age, birth weight and height, indication for PN, age at PN start, duration of PN, number of weekly PN infusions, weight-for-age and height-for-age (SD), body mass index (BMI, kg/m2) as well as blood and urine analyses at the time of Dual X-ray absorptiometry (DXA) measurements. All BMD values were adjusted to statural age which corresponds to the 50th percentile of height. Growth failure (height-for-age ≤ -2SD) and MBD (at least one BMD measurement ≤ -2SD) were analyzed according to the indication of PN, duration of PN and PN dependency index (PNDI) by comparing means and performing logistic regression analysis. PNDI is the ratio of non-protein energy intake in HPN to resting energy expenditure using Schofield equations. RESULTS: Forty children were assessed at 12.4 ± 4.5 years of age. Mean age at PN start was 1.1 ± 3.6 y (median 0.5). The indications for PN were short bowel syndrome (SBS, n = 21), chronic intestinal pseudo-obstruction syndrome (CIPOS, n = 10) and congenital enteropathies (CE, n = 9). The mean number of PN perfusions was 6 ± 1/week. PNDI was 110 ± 30%. The mean serum level of 25-OHD3 was suboptimal at 26.5 ± 9.1 ng/mL (66.2 ± 22.8 nmol/L). The mean concentrations of calcium, phosphorus, and parathyroid hormone (PTH) were in the normal ranges. Eight children (20%) had PTH levels above normal with low 25-OHD3 levels. The mean weight-for-age and height-for-age Z-scores SDS were 0.4 ± 0.9 and -0.5 ± 1.1 respectively. The actual height was lower than genetic target height (p < 0.001). The BMD Z-scores, adjusted to the 50th percentile of height, of the spine, the left femur and the whole body were: -1.1 ± 1.7, -1.2 ± 1.5 and -1.5 ± 1.8 SDS respectively. Children with CE had significantly lower BMD values than those with SBS and CIPOS (p = 0.01). Only two children had bone fractures after a mild trauma (5%). CONCLUSIONS: All children on long-term PN, are at risk of low BMD. High dependency on PN (PNDI>120%) and very long-term PN (>10 years) do not appear to increase the risk of growth failure nor MBD. PN-related bone fractures were rare. Close follow-up remains mandatory.
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Enfermedades Óseas Metabólicas/complicaciones , Enfermedades Intestinales/complicaciones , Nutrición Parenteral en el Domicilio/métodos , Nutrición Parenteral en el Domicilio/estadística & datos numéricos , Densidad Ósea/fisiología , Enfermedades Óseas Metabólicas/fisiopatología , Niño , Estudios Transversales , Femenino , Francia , Humanos , Enfermedades Intestinales/fisiopatología , Masculino , Estudios Retrospectivos , Factores de RiesgoRESUMEN
INTRODUCTION: Hepatitis E virus (HEV) is a major cause of acute viral hepatitis worldwide, usually asymptomatic in children. However, a growing number of publications over the last decade have documented cases of chronic hepatitis related to HEV-genotype 3 infection, and progressing to cirrhosis in immuno-compromised patients, particularly in adult kidney transplant recipients. The aim of our study was to evaluate the prevalence and severity of HEV infection among pediatric liver transplant (PLT) recipients managed in our center. MATERIAL AND METHODS: Between November 1st 2014 and January 1st 2016, PLT recipients (less than 18 years-old) were screened for HEV infection [determined by HEV serology, HEV- immunoglobulin M (IgM) and immunoglobulin G (IgG), and HEV-ribonucleic acid (RNA) by reverse transcriptase polymerase chain reaction] at their annual follow-up visit. RESULTS: Eighty children were tested for HEV infection a mean of 5.4±5.3 years after liver transplantation (LT). The main indication for LT was biliary atresia (n=47, 59%). The prevalence of HEV-IgG was 8% (n=6; age range 1.3 to 14.2 years-old at the time of HEV testing). Prevalence increased to 30% when considering only the 20 children with a past history of an unexplained episode of elevated transaminases since LT. None had HEV IgM, serum HEV-RNA, or increased transaminases at the time of HEV testing. Among the six IgG seropositive children, two had received intravenous immunoglobulins prior to screening and four children had a negative control (seroreversion) 3 to 42 months after the first testing. CONCLUSION: The prevalence of HEV infection in our cohort is low and similar to other pediatric reports. We saw no cases of chronic hepatitis or fibrosis attributable to HEV. The lower immunosuppressive regimen used in PLT children compared to other solid organ transplant recipients may account for this good outcome.
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Hepatitis E/epidemiología , Trasplante de Hígado , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/virología , Adolescente , Niño , Preescolar , Femenino , Francia/epidemiología , Anticuerpos Antihepatitis/sangre , Hepatitis E/sangre , Virus de la Hepatitis E/inmunología , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/sangre , Prevalencia , Estudios Seroepidemiológicos , Índice de Severidad de la EnfermedadRESUMEN
Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.
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Enterocolitis/tratamiento farmacológico , Enterocolitis/genética , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirazoles/uso terapéutico , Factor de Transcripción STAT3/genética , Enfermedad Crónica , Diarrea/etiología , Enterocolitis/complicaciones , Femenino , Mutación con Ganancia de Función , Humanos , Nitrilos , Pirimidinas , Secuenciación del Exoma , Adulto JovenRESUMEN
BACKGROUND: Infants with intestinal failure (IF) are at increased risk of malnutrition and require adapted nutrition support. Optimal weight gain during nutrition rehabilitation should occur at the velocity of statural age (adjusted to the 50th percentile of height) and not chronological age. The aim of this study was to assess the relationship between weight gain during catch-up growth, nonprotein energy intake (NPEI) provided by total parenteral nutrition (TPN), and resting energy expenditure (REE) in children with severe malnutrition due to IF. MATERIALS AND METHODS: This retrospective study included all infants with severe malnutrition treated with TPN for IF between January 1, 2010, and December 31, 2013. They all had no or minimal oral intake (<10% REE). The REE was calculated using the Schofield equations. RESULTS: Seventeen children were included (11 boys) with a mean age at TPN onset of 5 mo. They were followed for a mean duration of 39 days. On admission, body weight and height were -3.1 ± 0.9 and -3.3 ± 1.3 SD, respectively. The indications for TPN were short bowel syndrome (n = 10), congenital enteropathy (microvillous inclusion disease, n = 6) and chronic intestinal pseudo-obstruction syndrome (n = 1). After 28 days of nutrition rehabilitation with full NPEI from TPN, the observed weight gain was 110 ± 5% of optimal weight gain for statural age. The mean NPEI from TPN was 104.3 ± 8.0 kcal/kg/d. The mean ratio of NPEI over REE was 2.1 ± 0.2. CONCLUSION: Optimal weight gain was achieved with NPEI from TPN twice the REE in severely malnourished infants with IF. NPEI values were adequate and not excessive for age.
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Trastornos de la Nutrición del Lactante/terapia , Nutrición Parenteral Total/métodos , Aumento de Peso/fisiología , Femenino , Humanos , Lactante , Trastornos de la Nutrición del Lactante/fisiopatología , Masculino , Estudios RetrospectivosRESUMEN
Abstract Background: Difficult or impossible face mask ventilation complicated with difficult tracheal intubation during anesthesia induction occurs in 0.4% of adult anesthesia cases, possibly leading to life-threatening complications. Because of such catastrophes, muscle relaxants have been recommended to be administered after confirming adequate face mask ventilation without a solid scientific validation of this principal. Methods: In this observational study, the ease of ventilation and the scores of direct laryngoscopy views before and after administration of cisatracurium were assessed in ninety young healthy adults, without anesthetic risks and without foreseen difficult intubation and who were scheduled for general elective surgeries. Results: Before muscle relaxation, 43 patients (48%) were Cormack Grade I, while the remaining 47 patients (52%) were either Cormack Grade II (28 patients, 31%) or Cormack Grade II (19 patients, 21%). Following muscle relaxation with cisatracurium, the number of patients with Cormack Grade I significantly increased from 43 patients (48%) to 65 patients (72%) (p = 0.0013). Only 1 patient out of 19 patients (5%) improved his Cormack grade from Grade III to Grade I while 16 out 19 patients (84%) improved their Cormack grade from Grade III to Grade II after the use of cisatracurium. The quality of face mask ventilation did not differ with and without muscle relaxants in all patients. Conclusion: The use of cisatracurium in healthy young adults undergoing general elective surgeries with no anticipated difficult endotracheal intubation had no effect on the quality of face mask ventilation despite resulting in a quantifiable improvement in the laryngeal view.
Resumo Justificativa: A ventilação difícil ou impossível via máscara facial complicada pela intubação traqueal difícil durante a indução da anestesia ocorre em 0,4% dos casos de anestesia em adultos, possivelmente leva a complicações fatais. Devido a tais catástrofes, recomendou-se que a administração de relaxantes musculares seja feita após a confirmação de ventilação adequada via máscara facial, sem uma validação científica sólida dessa conduta. Métodos: Neste estudo observacional, a facilidade de ventilação e os escores de visibilidade em laringoscopia direta antes e após a administração de cisatracúrio foram avaliados em 90 adultos jovens e saudáveis, sem riscos anestésicos e sem intubação difícil prevista, agendados para cirurgias eletivas gerais. Resultados: Antes do relaxamento muscular, 43 pacientes (48%) eram Cormack Grau I, enquanto os 47 (52%) restantes eram ou Cormack Grau II (28, 31%) ou Cormack Grade III (19, 21%). Após o relaxamento muscular com cisatracúrio, o número de pacientes com Cormack Grau I aumentou significativamente de 43 (48%) para 65 (72%) (p = 0,0013). Apenas um paciente (5%) dos 19 melhorou sua classificação de Cormack do Grau III para o Grau I, enquanto 16 dos 19 (84%) melhoraram suas classificações de Cormack do Grau III para o grau II após o uso de cisatracúrio. A qualidade da ventilação via máscara facial não diferiu com ou sem relaxantes musculares em todos os pacientes. Conclusão: O uso de cisatracúrio em adultos jovens saudáveis submetidos a cirurgias eletivas gerais sem intubação traqueal difícil prevista não teve efeito sobre a qualidade da ventilação via máscara facial, mesmo resultando em melhora quantificável da visibilidade da laringe.
Asunto(s)
Humanos , Masculino , Femenino , Adolescente , Adulto , Adulto Joven , Fármacos Neuromusculares no Despolarizantes/uso terapéutico , Anestesia/métodos , Laringoscopía/métodos , Estudios Prospectivos , Persona de Mediana EdadRESUMEN
BACKGROUND: Difficult or impossible face mask ventilation complicated with difficult tracheal intubation during anesthesia induction occurs in 0.4% of adult anesthesia cases, possibly leading to life-threatening complications. Because of such catastrophes, muscle relaxants have been recommended to be administered after confirming adequate face mask ventilation without a solid scientific validation of this principal. METHODS: In this observational study, the ease of ventilation and the scores of direct laryngoscopy views before and after administration of cisatracurium were assessed in ninety young healthy adults, without anesthetic risks and without foreseen difficult intubation and who were scheduled for general elective surgeries. RESULTS: Before muscle relaxation, 43 patients (48%) were Cormack Grade I, while the remaining 47 patients (52%) were either Cormack Grade II (28 patients, 31%) or Cormack Grade II (19 patients, 21%). Following muscle relaxation with cisatracurium, the number of patients with Cormack Grade I significantly increased from 43 patients (48%) to 65 patients (72%) (p=0.0013). Only 1 patient out of 19 patients (5%) improved his Cormack grade from Grade III to Grade I while 16 out 19 patients (84%) improved their Cormack grade from Grade III to Grade II after the use of cisatracurium. The quality of face mask ventilation did not differ with and without muscle relaxants in all patients. CONCLUSION: The use of cisatracurium in healthy young adults undergoing general elective surgeries with no anticipated difficult endotracheal intubation had no effect on the quality of face mask ventilation despite resulting in a quantifiable improvement in the laryngeal view.
Asunto(s)
Anestesia , Laringoscopía/métodos , Fármacos Neuromusculares no Despolarizantes/uso terapéutico , Adolescente , Adulto , Anestesia/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Adulto JovenRESUMEN
BACKGROUND: Research in the field of informational needs of breast cancer patients is scarce. In the few published articles, these needs were usually not satisfied. The main objective of this study was to evaluate satisfaction regarding informational needs in women with breast cancer. The long-term goal was to guide physician-patient communication to meet these needs. MATERIALS AND METHODS: A survey with 21 questions was completed by 84 female patients receiving chemotherapy in a one-day hospital in Beirut, Lebanon. All patients were aware of their disease and agreed to participate in the survey. RESULTS: The doctor was the major source of information for patients followed by media (radio and television). The level of knowledge of patients concerning their disease was proportional to the number of information sources. Women aged younger than 45 years, diagnosed during the last three months before the survey and certified from high school were less satisfied with information given by the oncologist. The missing information was in relation with the steps of the treatment after the chemotherapy regimen, the risk of a family member (sisters and daughters) of developing the disease and management of lymphedema. CONCLUSIONS: This study generated a scale for the degree of satisfaction of information received by women with breast cancer from their oncologist. The physician can use this scale to improve his or her skills of communication to patients and diminish their level of fear and anxiety.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias de la Mama/tratamiento farmacológico , Neoplasias de la Mama/psicología , Evaluación de Necesidades , Educación del Paciente como Asunto , Pacientes/psicología , Adulto , Anciano , Femenino , Estudios de Seguimiento , Humanos , Persona de Mediana Edad , Relaciones Médico-Paciente , Proyectos Piloto , Pronóstico , Estrés PsicológicoRESUMEN
BACKGROUND: Parenteral nutrition (PN) is the main treatment for intestinal failure. OBJECTIVE: We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center. DESIGN: We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs). We compared our results to a previous study that was performed in our unit from 1980 to 2000 and included 302 patients. RESULTS: A total of 251 patients were included: 217 (86%) had a PDD. The mean ± SD age at HPN onset was 0.7 ± 0.3 y, with a mean duration of 1.9 ± 0.4 y. The indications for HPN were short bowel syndrome (SBS) (59%), PNDD (14%), congenital enteropathies (10%), chronic intestinal pseudo-obstruction syndromes (9%), inflammatory bowel diseases (5%), and other digestive diseases (3%). By 31 December 2013, 52% of children were weaned off of HPN, 9% of the PDD subgroup had intestinal transplantation, and 10% died mostly because of immune deficiency. The major complications of HPN were catheter-related bloodstream infections (CRBSIs) (1.7/1000 d of PN) and intestinal failure-associated liver disease (IFALD) (51 children; 20% of cohort). An increased rate of CRBSIs was observed compared with our previous study, but we saw a decreasing trend since 2012. No noteworthy deceleration of growth was observed in SBS children 6 mo after weaning off HPN. CONCLUSIONS: SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.
Asunto(s)
Nutrición Parenteral en el Domicilio , Infecciones Relacionadas con Catéteres/epidemiología , Femenino , Estudios de Seguimiento , Francia , Humanos , Lactante , Enfermedades Intestinales/terapia , Hepatopatías/epidemiología , Masculino , Nutrición Parenteral en el Domicilio/efectos adversos , Pronóstico , Estudios Retrospectivos , Síndrome del Intestino Corto/terapia , Resultado del TratamientoRESUMEN
STUDY OBJECTIVE: To evaluate the effect of clonidine when added to local anesthetics on duration of postoperative analgesia during retrobulbar block. DESIGN: Prospective, randomized controlled trial. SETTING: Operating room and Postanesthesia Care Unit of a university-affiliated hospital. SUBJECTS: 80 ASA physical status 1, 2, and 3 patients undergoing vitreoretinal surgery with or without scleral buckling. INTERVENTIONS: Patients in the control group (n = 40) received a retrobulbar block with 4.5 mL of lidocaine-bupivacaine and 0.5 mL of saline. Clonidine group patients (n = 40) received 4.5 mL of lidocaine-bupivacaine and 0.5 µg/kg of clonidine in a 0.5 mL volume. MEASUREMENTS: The time to first analgesic request, frequency of postoperative pain, and number of postoperative analgesic requests per patient were assessed. MAIN RESULTS: 37 patients in the control group (92.5%) versus 24 patients (60%) in the clonidine group reported pain postoperatively (P = 0.001), with a shorter time to first analgesic request noted in the control group (4.9 ± 3 vs 11.9 ± 5.3 hrs; P < 0.001). The median number of postoperative analgesic requests per patient during the first 24 hours was higher in the control group than the clonidine group [2 (0-3) vs. 1 (0-3); P < 0.001]. CONCLUSIONS: The addition of clonidine 0.5 µg/kg to the local anesthetics of a retrobulbar block for vitreoretinal surgery decreases the frequency of postoperative pain and prolongs the time of analgesia.