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1.
Pediatr Res ; 95(7): 1749-1753, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38280953

RESUMEN

BACKGROUND: The microbiological safety of donor milk (DM) is commonly ensured by Holder pasteurization (HoP, 62.5 °C for 30 min) in human milk banks despite its detrimental effects on bioactive factors. We compared the antimicrobial properties of DM after Holder pasteurization treatment or High Hydrostatic Pressure processing (HHP, 350 MPa at 38 °C), a non-thermal substitute for DM sterilization. METHODS: We assessed lactoferrin and lysozyme concentrations in raw, HHP- and HoP-treated pools of DM (n = 8). The impact of both treatments was evaluated on the growth of Escherichia coli and Group B Streptococcus in comparison with control media (n = 4). We also addressed the effect of storage of HHP treated DM over a 6-month period (n = 15). RESULTS: HHP milk demonstrated similar concentrations of lactoferrin compared with raw milk, while it was significantly decreased by HoP. Lysozyme concentrations remained stable regardless of the condition. Although a bacteriostatic effect was observed against Escherichia coli at early timepoints, a sharp bactericidal effect was observed against Group B Streptococcus. Unlike HoP, these results were significant for HHP compared to controls. Stored DM was well and safely preserved by HHP. CONCLUSION: Our study demonstrates that this alternative sterilization method shows promise for use with DM in human milk banks. IMPACT: Antimicrobial activity of donor milk after High Hydrostatic Pressure treatment has not been clearly evaluated. Donor milk lactoferrin is better preserved by High Hydrostatic Pressure than conventional Holder pasteurization, while lysozyme concentration is not affected by either treatment. As with Holder pasteurization, High Hydrostatic Pressure preserves donor milk bacteriostatic activity against E. coli in addition to bactericidal activity against Group B Streptococcus. Donor milk treated by High Hydrostatic Pressure can be stored safely for 6 months.


Asunto(s)
Escherichia coli , Presión Hidrostática , Lactoferrina , Bancos de Leche Humana , Leche Humana , Muramidasa , Pasteurización , Pasteurización/métodos , Leche Humana/química , Humanos , Muramidasa/análisis , Escherichia coli/crecimiento & desarrollo , Lactoferrina/análisis , Esterilización/métodos , Streptococcus agalactiae , Microbiología de Alimentos
2.
Artículo en Inglés | MEDLINE | ID: mdl-38899575

RESUMEN

INTRODUCTION: Pasteurized human donor milk (DM) is frequently used for feeding preterm newborns and extrauterine growth-restricted (EUGR) infants. Most human milk banks performed a pasteurization of DM using the standard method of Holder pasteurization (HoP) which consists of heating milk at 62.5°C for 30 min. High hydrostatic pressure (HHP) processing was proposed to be an innovative nonthermal method to pasteurize DM. However, the effect of different modes of DM pasteurization on body growth, intestinal maturation, and microbiota has never been investigated in vivo during the lactation. OBJECTIVES: We aimed to study these effects in postnatally growth-restricted (PNGR) mice pups daily supplemented with HoP-DM or HHP-DM. METHODS: PNGR was induced by increasing the number of pups per litter (15 pups/mother) at postnatal Day 4 (PND4). From PND8 to PND20, mice pups were supplemented with HoP-DM or HHP-DM. At PND21, the intestinal permeability was measured in vivo, the intestinal mucosal histology, gut microbiota, and short-chain fatty acids (SCFAs) level were analyzed. RESULTS: HHP-DM pups displayed a significantly higher body weight gain than HoP-DM pups during lactation. At PND21, these two types of human milk supplementations did not differentially alter intestinal morphology and permeability, the gene-expression level of several mucosal intestinal markers, gut microbiota, and the caecal SCFAs level. CONCLUSION: Our data suggest that HHP could be an attractive alternative to HoP and that HHP-DM may ensure a better body growth of preterm and/or EUGR infants.

3.
J Pediatr ; 251: 134-139.e2, 2022 12.
Artículo en Inglés | MEDLINE | ID: mdl-35853483

RESUMEN

OBJECTIVE: To compare the efficacy of, and complications from, the 2 main treatments for achalasia: endoscopic dilatation and surgical cardiomyotomy (Heller's myotomy). STUDY DESIGN: We retrospectively collected data on children treated for achalasia over an 11-year period from 8 tertiary pediatric centers. A line of treatment was defined as performing either Heller's myotomy or 1-3 sessions of endoscopy dilatation over 3 months. Treatment success was a priori defined as clinical improvement and no need for new treatment. RESULTS: Ninety-seven children (median age, 12 years; 57% boys) were included. The median time to diagnosis was 10.5 months, and the median follow-up period was 27 months. Thirty-seven children were treated by Heller's myotomy and 60 by endoscopy dilatation as the first-line treatment. After adjustment for potentially confounding factors, Heller's myotomy was significantly more successful than endoscopy dilatation (hazard ratio, 3.93 [1.74; 8.88]; P = .001), with a median survival without failure of 49 and 7 months, respectively, and with no significant difference in the occurrence of complications (35.2% for Heller's myotomy, 29.7% for endoscopy dilatation, P = .56). Hydrostatic dilatation was as successful as pneumatic dilatation (hazard ratio, 1.35 [0.56; 3.23]; P = .50). CONCLUSIONS: Heller's myotomy is more successful than endoscopy dilatation, with no significant difference in the occurrence of serious complications. This raises the potential role of peroral endoscopic myotomy as an alternative treatment to Heller's myotomy.


Asunto(s)
Acalasia del Esófago , Miotomía de Heller , Niño , Masculino , Humanos , Femenino , Acalasia del Esófago/cirugía , Dilatación , Estudios Retrospectivos , Endoscopía
4.
BMC Infect Dis ; 17(1): 452, 2017 06 26.
Artículo en Inglés | MEDLINE | ID: mdl-28651525

RESUMEN

BACKGROUND: Respiratory syncytial virus (RSV) is responsible for severe respiratory infections and higher costs in medical care. The two aims of this work were to assess the performances of SOFIA®RSV tests in "real-life-laboratory" conditions (study 1) and implemented at point-of-care testing in a pediatric emergency department (ED, study 2), during two consecutive winter seasons. METHODS: In study 1, fresh nasopharyngeal swabs from patients of all ages were sampled in 1.5 ml of Universal virological Transport Medium (UTM) and prospectively tested using SOFIA®RSV tests. In study 2, conducted in a pediatric ED, nasopharyngeal swabs were placed in 3 ml of UTM. All SOFIA®RSV tests were confirmed by molecular testing, considered as reference method. The epidemiological and clinical features of tested patients, as well as the care of these patients after obtaining quick results were evaluated. RESULTS: The sensitivities of SOFIA®RSV in infants (aged under 24 months) performed in the laboratory and in the pediatric ED were respectively 95% (95% CI: 86.8-98.1) and 74.8% (95% CI: 68.0-80.9) compared to PCR. In study 1, the sensitivity among children (from 2 to 15 years old) and adults (above 15 years old) dropped to 45% (95% CI: 23.1-68.5) and 59% (95% CI: 32.9-81.6), respectively. In study 2, there were some differences in bed-management of SOFIA®RSV positive compared to SOFIA®RSV negative infants. CONCLUSIONS: SOFIA®RSV tests performed in the laboratory and in the pediatric ED show high and satisfactory sensitivities among young children under 24 months, which supports its robustness and reliability. However, the impact of these tests on patient care at point-of-care cannot be clearly assessed when considering the limits of the study 2 design.


Asunto(s)
Pruebas Diagnósticas de Rutina , Infecciones por Virus Sincitial Respiratorio/diagnóstico , Adolescente , Adulto , Niño , Preescolar , Servicio de Urgencia en Hospital , Humanos , Lactante , Laboratorios , Medicina de Urgencia Pediátrica , Sistemas de Atención de Punto , Estudios Prospectivos , Reproducibilidad de los Resultados , Infecciones por Virus Sincitial Respiratorio/virología , Virus Sincitial Respiratorio Humano/patogenicidad , Estaciones del Año
5.
Nutrients ; 15(12)2023 Jun 16.
Artículo en Inglés | MEDLINE | ID: mdl-37375672

RESUMEN

BACKGROUND: An inadequate perinatal nutritional environment can alter the maturation of the intestinal barrier and promote long-term pathologies such as metabolic syndrome or chronic intestinal diseases. The intestinal microbiota seems to play a determining role in the development of the intestinal barrier. In the present study, we investigated the impact of consuming an early postnatal prebiotic fiber (PF) on growth, intestinal morphology and the microbiota at weaning in postnatal-growth-restricted mice (PNGR). METHODS: Large litters (15 pups/mother) were generated from FVB/NRj mice to induce PNGR at postnatal day 4 (PN4) and compared to control litters (CTRL, 8 pups/mother). PF (a resistant dextrin) or water was orally administered once daily to the pups from PN8 to PN20 (3.5 g/kg/day). Intestinal morphology was evaluated at weaning (PN21) using the ileum and colon. Microbial colonization and short-chain fatty acid (SCFA) production were investigated using fecal and cecal contents. RESULTS: At weaning, the PNGR mice showed decreased body weight and ileal crypt depth compared to the CTRL. The PNGR microbiota was associated with decreased proportions of the Lachnospiraceae and Oscillospiraceae families and the presence of the Akkermansia family and Enterococcus genus compared to the CTRL pups. The propionate concentrations were also increased with PNGR. While PF supplementation did not impact intestinal morphology in the PNGR pups, the proportions of the Bacteroides and Parabacteroides genera were enriched, but the proportion of the Proteobacteria phylum was reduced. In the CTRL pups, the Akkermansia genus (Verrucomicrobiota phylum) was present in the PF-supplemented CTRL pups compared to the water-supplemented ones. CONCLUSIONS: PNGR alters intestinal crypt maturation in the ileum at weaning and gut microbiota colonization. Our data support the notion that PF supplementation might improve gut microbiota establishment during the early postnatal period.


Asunto(s)
Suplementos Dietéticos , Prebióticos , Femenino , Embarazo , Animales , Ratones , Intestinos , Lactancia , Ratones Endogámicos
6.
Front Nutr ; 10: 1107054, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36891163

RESUMEN

The milk metabolome is composed of hundreds of molecules that can impact infant development. In preterm infants, sterilized donor milk (DM) is frequently used for their feeding. We aimed to identify differences in the metabolome of DM after two types of milk sterilization: the Holder pasteurization (HoP) and a high hydrostatic pressure (HP) processing. DM samples were sterilized by HoP (62.5°C for 30 min) or processed by HP (350 MPa at 38°C). 595 milk metabolites were analyzed using an untargeted metabolomic analysis. Both treatments differentially altered several classes of compounds. The major changes noted included decreased levels of free fatty acids, phospholipid metabolites, and sphingomyelins. Decreases were more strongly noted in HP samples rather than in HoP ones. Both HoP and HP treatments increased the levels of ceramides and nucleotide compounds. The sterilization of human milk altered its metabolome especially for lipids.

7.
Front Pediatr ; 11: 1120008, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37842027

RESUMEN

Sterilized donor milk (DM) is frequently used for feeding preterm infants. To date, the effect of different modes of DM sterilization on short-chain fatty acids (SCFAs) remains unknown. We aimed to quantify SCFAs in DM samples after two types of milk sterilization: the Holder pasteurization (HoP) and a high hydrostatic pressure (HP) processing. Eight pooled DM samples were sterilized by HoP (62.5°C for 30 min) or processed by HP (350 MPa at 38°C). Raw DM was used as control. Six SCFAs were quantified by gas chromatography/mass spectrometry. Compared to raw milk, both HoP and HP treatment did not significantly modulate the concentration of acetate, butyrate, propionate and isovalerate in DM. Valerate and isobutyrate were undetectable in DM samples. In conclusion, both HoP and HP processing preserved milk SCFAs at their initial levels in raw human milk.

8.
Nutrients ; 15(18)2023 Sep 18.
Artículo en Inglés | MEDLINE | ID: mdl-37764826

RESUMEN

BACKGROUND: Human milk banks (HMBs) provide sterilized donor milk (DM) for the feeding of preterm infants. Most HMBs use the standard method of Holder pasteurization (HoP) performed by heating DM at 62.5 °C for 30 min. High hydrostatic pressure (HHP) processing has been proposed as an alternative to HoP. This study aims to evaluate intestinal barrier integrity and microbiota composition in adult mice subjected to a chronic oral administration of HoP- or HHP-DM. METHODS: Mice were treated by daily gavages with HoP- or HHP-DM over seven days. Intestinal barrier integrity was assessed through in vivo 4 kDa FITC-dextran permeability assay and mRNA expression of several tight junctions and mucins in ileum and colon. Cecal short chain fatty acids (SCFAs) and microbiota were analyzed. RESULTS: HHP-DM mice displayed decreased intestinal permeability to FITC-dextran and increased ileal mRNA expression levels of two tight junctions (Ocln and Cdh1) and Muc2. In the colon, mRNA expression levels of two tight junctions (Cdh1 and Tjp1) and of two mucins (Muc2 and Muc4) were decreased in HHP-DM mice. Cecal SCFAs and microbiota were not different between groups. CONCLUSIONS: HHP processing of DM reinforces intestinal barrier integrity in vivo without affecting gut microbiota and SCFAs production. This study reinforces previous findings showing that DM sterilization through HHP might be beneficial for the intestinal maturation of preterm infants compared with the use of HoP for the treatment of DM.


Asunto(s)
Pasteurización , Recién Nacido , Adulto , Lactante , Humanos , Animales , Ratones , Leche Humana , Presión Hidrostática , Recien Nacido Prematuro , Esterilización , ARN Mensajero
10.
Food Chem ; 377: 131957, 2022 May 30.
Artículo en Inglés | MEDLINE | ID: mdl-34990954

RESUMEN

In human milk banks (HMBs), donor milk (DM) is commonly sterilized by Holder pasteurization (HoP). High hydrostatic pressure (HHP) processing is an innovative, alternative method for DM sterilization. We evaluated the impact of HHP processing on the concentration of seven metabolic milk hormones. Eight samples of raw DM were aliquoted. One aliquot was sterilized by HoP (62 °C for 30 min), and another was processed by HHP (350 MPa at 38 °C). Compared with raw DM, HoP milk displayed reduced concentrations of insulin, nesfatin-1, cortisol, leptin, apelin and GLP-1, though adiponectin levels were unchanged. HHP processing maintained the levels of insulin, nesfatin-1, cortisol and leptin at their initial levels in raw DM, reduced apelin and adiponectin levels, but increased GLP-1 level. Sterilization of DM by HHP thus preserves the main metabolic hormones in human milk, underlining the interest of this method for use in HMBs.


Asunto(s)
Bancos de Leche Humana , Leche Humana , Femenino , Humanos , Presión Hidrostática , Insulina , Pasteurización
11.
Front Pediatr ; 10: 849815, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35573968

RESUMEN

Objectives: Although transient elastography (TE) is the primary noninvasive method for assessing liver fibrosis, its use remains to be validated in children. This study aims to evaluate the agreement between two-dimensional ultrasound shear wave elastography (2D-SWE) and TE to assess pediatric liver stiffness method. Methods: During the 18-month study, we prospectively included 101 consecutive children (median age: 8.5 years, range: 1 month to 17 years) who required TE for medical reasons, and in whom 2D-SWE measurement was performed within a 3-month follow-up during a routine ultrasound. Liver elasticity values were classified according to the Metavir score using published pediatric norms for TE and according to the manufacturer's reference values for 2D-SWE. The Spearman's correlation coefficient was used to assess the relationship between the elasticity measured by the two techniques. Concordance was described by the Bland-Altman method. Results: A strong correlation (rho = 0.70, p < 0.001) was found between 2D-SWE and TE for the elasticity measures. The strength of correlation was higher among patients older than 6 years (rho = 0.79, p < 0.001). Concordance between liver fibrosis stages assessed by these techniques was moderate [weighted kappa = 0.46, (95% CI: 0.35-0.57)]. When considering stages over F2, 2D-SWE diagnostic performances showed a sensitivity of 85% (95% CI: 74-92) and a specificity of 57% (95% CI: 42-70) compared with TE. Conclusion: Measurements of the liver stiffness using 2D-SWE and TE are strongly correlated. The moderate concordance between these techniques for assessing the liver fibrosis stage provides evidence against alternating between these methods during follow-up of patients with the chronic liver diseases.

12.
Front Pediatr ; 10: 983717, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36120654

RESUMEN

Objectives: Malnutrition is common in children with biliary atresia (BA) awaiting liver transplantation (LT). Few studies have evaluated the effectiveness of enteral nutrition (EN) in these patients. The objective of this work was to assess the efficacy and tolerance of EN in children with BA awaiting LT. Methods: A total of 31 patients with BA followed between 1995 and 2018 were retrospectively included. Anthropometric indicators (weight, length, and head circumference) and adverse effects of EN were noted at the start (T0) and the end (T1) of EN. The z-scores for anthropometric indicators were compared between T0 and T1. Results: The median age at T0 was 7 months (interquartile range [IQR] 5-9), and the median duration of EN was 9 months (IQR 3-17). The z-scores for anthropometric variables improved from T0 to T1: -1.6 (IQR -2.5 to -1.0) to -0.5 (IQR -1.8 to 0.3) for median weight for age; -1.3 (IQR -2.4 to 0) to -0.4 (IQR -2.0 to 0.7) for length for age; -0.9 (IQR -2.3 to -0.3) to -0.3 (IQR -1.2 to 0.1) for weight for length; and -1.2 (IQR -2.1 to -0.6) to -0.2 (IQR -1.6 to 0.4) for body mass index (p < 0.05 for all comparisons). Nearly all (94%) of the patients had a weight-for-length z-score > -2 at the end of EN; 23% had adverse effects and 10% had complications leading to the cessation of EN. Conclusion: EN is effective and well tolerated in infants with BA awaiting LT.

13.
Clin Nutr ; 41(1): 1-8, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34861623

RESUMEN

BACKGROUND & AIMS: High hydrostatic pressure (HHP) processing is a non-thermal method proposed as an alternative to Holder pasteurization (HoP) for the treatment of human milk. HHP preserves numerous milk bioactive components that are degraded by HoP, but no data are available for milk oligosaccharides (HMOs) or the formation of Maillard reaction products, which may be deleterious for preterm newborns. METHODS: We evaluated the impact of HHP processing of human milk on 22 HMOs measured by liquid chromatography with fluorescence detection and on furosine, lactuloselysine, carboxymethyllysine (CML) and carboxyethyllysine (CEL) measured by liquid chromatography with tandem mass spectrometric detection (LC-MS/MS), four established indicators of the Maillard reaction. Human raw milk was sterilized by HoP (62.5 °C for 30 min) or processed by HHP (350 MPa at 38 °C). RESULTS: Neither HHP nor HoP processing affected the concentration of HMOs, but HoP significantly increased furosine, lactuloselysine, CML and CEL levels in milk. CONCLUSIONS: Our findings demonstrate that HPP treatment preserves HMOs and avoids formation of Maillard reaction products. Our study confirms and extends previous findings that HHP treatment of human milk provides safe milk, with fewer detrimental effects on the biochemically active milk components than HoP.


Asunto(s)
Manipulación de Alimentos/métodos , Productos Finales de Glicación Avanzada/síntesis química , Presión Hidrostática , Leche Humana/química , Oligosacáridos/química , Cromatografía Liquida , Humanos , Espectrometría de Masas en Tándem
14.
Front Pediatr ; 9: 719626, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34631620

RESUMEN

Objectives: Experience of hypnosis in gastrointestinal (GI) endoscopy is scarce in children. Our aims were to assess the rate of successful GI endoscopy performed using hypnosis alone or in combination with midazolam, with or without additional equimolar mixture of oxygen and nitrous oxide (EMONO), and to identify predictive factors of successful endoscopy in children. Methods: This prospective single-centre study included children older than 6 years requiring a diagnostic esophagogastroduodenoscopy (EGD) or rectosigmoidoscopy. Ericksonian hypnosis was performed alone or in combination with midazolam, with or without additional EMONO. Successful endoscopy was defined by a complete and well-tolerated procedure. Levels of satisfaction of the endoscopist, nurse, and patient were assessed. Results: One hundred forty children [70 boys, median age: 12 years (Q1-Q3: 9-14)] were included over a 14-month period. They underwent EGD in 51.4% (n = 72) and rectosigmoidoscopy in 48.6% (n = 68) of cases. EMONO and midazolam were combined with hypnosis in 136 cases (97.1%). Successful endoscopy rate reached 82.9%. The procedure was interrupted due to poor tolerance and was rescheduled under general anaesthesia in 11 patients (7.9%). Predictive factors for successful endoscopy were older age (13 vs. 8 years, OR: 1.34, CI 95% [1.10-1.62], p = 0.003) and type of endoscopy (EGD vs. rectosigmoidoscopy, OR: 16.34 [2.14-124.68], p = 0.007). A good cooperation of the patient was reported by the endoscopist and the nurse in 88.4 and 86.9% of cases, respectively. Ninety-two per cent of patients mentioned that the procedure went well. Conclusions: Our study suggests that hypnosis combined with EMONO and/or midazolam is of additional value to perform diagnostic EGD or rectosigmoidoscopy in children older than 6 years without systematic need for general anaesthesia.

15.
Dig Liver Dis ; 53(10): 1268-1275, 2021 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-34187767

RESUMEN

BACKGROUND: Chronic abdominal pain occurs frequently in pediatric patients with inflammatory bowel disease (IBD) in remission. AIMS: To assess the prevalence and factors associated with Functional Abdominal Pain Disorders among IBD children in remission (IBD-FAPD). METHODS: Patients with IBD for > 1 year, in clinical remission for ≥ 3 months were recruited from a National IBD network. IBD-FAPDs were assessed using the Rome III questionnaire criteria. Patient- or parent- reported outcomes were assessed. RESULTS: Among 102 included patients, 57 (56%) were boys, mean age (DS) was 15.0 (± 2.0) years and 75 (74%) had Crohn's disease. Twenty-two patients (22%) had at least one Functional Gastrointestinal Disorder among which 17 had at least one IBD-FAPD. Past severity of disease or treatments received and level of remission were not significantly associated with IBD-FAPD. Patients with IBD-FAPD reported more fatigue (peds-FACIT-F: 35.9 ± 9.8 vs. 43.0 ± 6.9, p = 0.01) and a lower HR-QoL (IMPACT III: 76.5 ± 9.6 vs. 81.6 ± 9.2, p = 0.04) than patients without FAPD, and their parents had higher levels of State and Trait anxiety than the other parents. CONCLUSIONS: Prevalence of IBD-FAPD was 17%. IBD-FAPD was not associated with past severity of disease, but with fatigue and lower HR-QoL.


Asunto(s)
Dolor Abdominal/etiología , Enfermedades Inflamatorias del Intestino/complicaciones , Calidad de Vida , Dolor Abdominal/psicología , Adolescente , Estudios de Casos y Controles , Estudios Transversales , Fatiga/etiología , Fatiga/psicología , Femenino , Humanos , Enfermedades Inflamatorias del Intestino/psicología , Masculino , Padres/psicología , Medición de Resultados Informados por el Paciente , Inducción de Remisión , Índice de Severidad de la Enfermedad
16.
Clin Nutr ESPEN ; 29: 119-124, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30661674

RESUMEN

BACKGROUND & AIMS: Children on home parenteral nutrition and their parents not only engage with complex nutritional issues but also have to manage difficult social and financial problems with social and clinical support that may not always meet their needs. Baxter's HPN-QOL questionnaire, assesses the QOL of adult patients treated with HPN, and has been developed rigorously using standard guidelines, measuring various dimensions of QOL. Our aim was to use this tool to explore how HPN influences the QOL of paediatric patients. METHODS: The HPN-QOL questionnaire was modified to suit a paediatric HPN population. Data on demographics, aetiology of intestinal failure and duration of HPN were collected from a departmental database. Quality-of-Life grading of functional and symptom scales, HPN specific items and overall QOL Numerical Rating Scales were determined. RESULTS: Fourteen out of 17 families returned the completed questionnaires. QOL was significantly impaired by increased dependency regarding items of daily living such as eating, dressing, washing, and mobility, but was not affected in the domains of school attendance, general fatigue, pain and body image. There were no significant differences in QOL when patients with and without enterostomy were compared. Patients felt well supported by the hospital nutrition team in managing logistics related to HPN. CONCLUSIONS: QOL in HPN patients was not significantly affected by the medical aspects of care. This descriptive study highlights the need for further integration of medical and social care in order to support families of children receiving HPN as QOL was impaired in relation to activities of daily living and social functioning.


Asunto(s)
Nutrición Parenteral en el Domicilio , Calidad de Vida , Actividades Cotidianas , Adolescente , Niño , Preescolar , Estudios Transversales , Enterostomía , Femenino , Hospitales , Humanos , Lactante , Enfermedades Intestinales , Intestino Delgado , Masculino , Estado Nutricional , Nutrición Parenteral en el Domicilio/psicología , Proyectos Piloto , Síndrome del Intestino Corto , Encuestas y Cuestionarios
17.
J Bone Miner Res ; 33(8): 1480-1488, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29693731

RESUMEN

Pseudohypoparathyroidism type 1A (PHP1A), pseudoPHP (PPHP), and PHP type 1B (PHP1B) are caused by maternal and paternal GNAS mutations and abnormal methylation at maternal GNAS promoter(s), respectively. Adult PHP1A patients are reportedly obese and short, whereas most PPHP patients are born small. In addition to parathyroid hormone (PTH) resistance, PHP1A and PHP1B patients may display early-onset obesity. Because early-onset and severe obesity and short stature are daily burdens for PHP1A patients, we aimed at improving knowledge on the contribution of the GNAS transcripts to fetal and postnatal growth and fat storage. Through an international collaboration, we collected growth and weight data from birth until adulthood for 306 PHP1A/PPHP and 220 PHP1B patients. PHP1A/PPHP patients were smaller at birth than healthy controls, especially PPHP (length Z-score: PHP1A -1.1 ± 1.8; PPHP -3.0 ± 1.5). Short stature is observed in 64% and 59% of adult PHP1A and PPHP patients. PHP1B patients displayed early postnatal overgrowth (height Z-score at 1 year: 2.2 ± 1.3 and 1.3 ± 1.5 in autosomal dominant and sporadic PHP1B) followed by a gradual decrease in growth velocity resulting in normal adult height (Z-score for both: -0.4 ± 1.1). Early-onset obesity characterizes GNAS alterations and is associated with significant overweight and obesity in adults (bodey mass index [BMI] Z-score: 1.4 ± 2.6, 2.1 ± 2.0, and 1.4 ± 1.9 in PPHP, PHP1A, and PHP1B, respectively), indicating that reduced Gsα expression is a contributing factor. The growth impairment in PHP1A/PPHP may be due to Gsα haploinsufficiency in the growth plates; the paternal XLαs transcript likely contributes to prenatal growth; for all disease variants, a reduced pubertal growth spurt may be due to accelerated growth plate closure. Consequently, early diagnosis and close follow-up is needed in patients with GNAS defects to screen and intervene in case of early-onset obesity and decreased growth velocity. © 2018 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research (ASBMR).


Asunto(s)
Desarrollo Óseo/genética , Cromograninas/genética , Epigénesis Genética , Subunidades alfa de la Proteína de Unión al GTP Gs/genética , Sitios Genéticos , Predisposición Genética a la Enfermedad , Obesidad/genética , Adulto , Edad de Inicio , Índice de Masa Corporal , Femenino , Humanos , Recién Nacido , Masculino , Embarazo , Seudohipoparatiroidismo/genética , Aumento de Peso/genética
18.
Rev Prat ; 66(9): 966-967, 2016 Nov.
Artículo en Francés | MEDLINE | ID: mdl-30512360

RESUMEN

Bronchiolitis epidemiology. Respiratory syncytial virus is the most important viral pathogen causing acute lower respiratory infection and infants hospitalizations. Other viruses are also identified (métapneumovirus, rhino/enterovirus, coronavirus, influenza, parainfluenza, adenovirus …). The research develops on new therapies and prevention.


Épidémiologie de la bronchiolite aiguë. La bronchiolite à virus respiratoire syncytial est chez le nourrisson la cause la plus fréquente de détresse respiratoire et d'hospitalisation. D'autres virus sont également identifiés (métapneumovirus, rhino/entérovirus, coronavirus, virus para-influenza et influenza, adénovirus…). Les recherches se développent sur la prévention et de nouvelles thérapeutiques.

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