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BACKGROUND AND AIMS: To systematically review the literature for reports on Wolcott-Rallison syndrome, focusing on the spectrum and natural history, genotype-phenotype correlations, patient and native liver survival, and long-term outcomes. METHODS: PubMed, Livio, Google Scholar, Scopus and Web of Science databases were searched. Data on genotype, phenotype, therapy, cause of death and follow-up were extracted. Survival and correlation analyses were performed. RESULTS: Sixty-two studies with 159 patients met the inclusion criteria and additional 30 WRS individuals were collected by personal contact. The median age of presentation was 2.5 months (IQR 2) and of death was 36 months (IQR 50.75). The most frequent clinical feature was neonatal diabetes in all patients, followed by liver impairment in 73%, impaired growth in 72%, skeletal abnormalities in 59.8%, the nervous system in 37.6%, the kidney in 35.4%, insufficient haematopoiesis in 34.4%, hypothyroidism in 14.8% and exocrine pancreas insufficiency in 10.6%. Episodes of acute liver failure were frequently reported. Liver transplantation was performed in six, combined liver-pancreas in one and combined liver-pancreas-kidney transplantation in two individuals. Patient survival was significantly better in the transplant cohort (p = .0057). One-, five- and ten-year patient survival rates were 89.4%, 65.5% and 53.1%, respectively. Liver failure was reported as the leading cause of death in 17.9% of cases. Overall survival was better in individuals with missense mutations (p = .013). CONCLUSION: Wolcott-Rallison syndrome has variable clinical courses. Overall survival is better in individuals with missense mutations. Liver- or multi-organ transplantation is a feasible treatment option to improve survival.
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Epífisis , Trasplante de Hígado , Osteocondrodisplasias , Humanos , Osteocondrodisplasias/genética , Epífisis/anomalías , Epífisis/cirugía , Estudios de Seguimiento , Lactante , Insuficiencia Pancreática Exocrina/genética , Diabetes Mellitus/genética , Preescolar , Fallo Hepático Agudo/genética , Fallo Hepático Agudo/mortalidad , Fallo Hepático Agudo/cirugía , Hipotiroidismo/genética , Fenotipo , Estudios de Asociación Genética , Diabetes Mellitus Tipo 1 , eIF-2 QuinasaRESUMEN
AIM: To evaluate the pattern, impact on quality of life and management of common functional gastrointestinal disorders (FGIDs) and related signs and symptoms in infants below 1 year of age in Africa. METHODS: Multicentre, cross-sectional, observational study in 10 African countries. At the first paediatric consultation of children with gastrointestinal symptoms, the perception of paediatricians on FGIDs (infant colic, constipation and regurgitation) and gas/bloating, impact on infant quality of life and parental anxiety and patient management practices were evaluated by standardised questionnaires. RESULTS: Questionnaires were completed by 759 paediatricians for 10 812 infants. Overall, 49.9% of paediatricians reported ≥30% of first infant consultations each month for FGIDs or related symptoms. Infant colic was most commonly diagnosed (57.6% of infants), followed by gas/bloating (43.2%), regurgitation (39.7%) and constipation (31.4%). Overall, 53% presented >1 symptom. Mean scores for infant quality of life, sleep and parental anxiety were worse when children had multiple symptoms compared to children with a single symptom (P < .025). Prescription of medication was common (62.4%). There were no consistent differences between countries. CONCLUSION: Functional gastrointestinal disorder occurrence in Africa was high with a gap between expert recommendation that emphasises parental reassurance and nutritional advice and daily practice, particularly prescription of medication.
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Enfermedades Gastrointestinales , Calidad de Vida , África , Niño , Estudios Transversales , Enfermedades Gastrointestinales/diagnóstico , Enfermedades Gastrointestinales/epidemiología , Enfermedades Gastrointestinales/terapia , Humanos , Lactante , PrevalenciaRESUMEN
OBJECTIVES: The Bristol Stool Form Scale (BSFS) is inadequate for non-toilet trained children. The Brussels Infant and Toddler Stool Scale (BITSS) was developed, consisting of 7 photographs of diapers containing stools of infants and toddlers. We aimed to evaluate interobserver reliability of stool consistency assessment among parents, nurses, and medical doctors (MDs) using the BITSS. METHODS: In this multicenter cross-sectional study (2016-2017), BITSS photographs were rated according to the BSFS. The reliability of the BITSS was evaluated using the overall proportion of perfect agreement and the linearly weighted κ statistic. RESULTS: A total of 2462 observers participated: 1181 parents (48.0%), 624 nurses (25.3%), and 657 MDs (26.7%). The best-performing BITSS photographs corresponded with BSFS type 7 (87.5%) and type 4 (87.6%), followed by the BITSS photographs representing BSFS type 6 (75.0%), BSFS type 5 (68.0%), BSFS type 1 (64.8%), and BSFS type 3 (64.6%). The weakest performing BITSS photograph corresponded with BSFS type 2 (49.7%). The overall weighted κ-value was 0.72 (95% CI 0.59-0.85; good agreement). Based on these results, photographs were categorized per stool group as hard (BSFS type 1-3), formed (BSFS type 4), loose (BSFS types 5 and 6), or watery (BSFS type 7) stools. According to this new categorization system, correct allocation for each photograph ranged from 83 to 96% (average: 90%). The overall proportion of correct allocations was 72.8%. CONCLUSIONS: BITSS showed good agreement with BSFS. Using the newly categorized BITSS photographs, the BITSS is reliable for the assessment of stools of non-toilet trained children in clinical practice and research. A multilanguage translated version of the BITSS can be downloaded at https://bitss-stoolscale.com/.
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Heces , Enfermedades Gastrointestinales/diagnóstico , Fotograbar/estadística & datos numéricos , Escala Visual Analógica , Bélgica , Estudios Transversales , Femenino , Humanos , Lactante , Masculino , Enfermeras y Enfermeros/estadística & datos numéricos , Variaciones Dependientes del Observador , Padres , Médicos/estadística & datos numéricos , Reproducibilidad de los ResultadosRESUMEN
BACKGROUND: Iron deficiency (ID) with or without anemia is associated with impaired mental and psychomotor development. Given the paucity of information on physicians' knowledge and practices on iron (Fe) supplementation and impact of ID in the Middle East and North Africa, it was felt important to conduct a survey. METHOD: A group of expert physicians developed a questionnaire that was randomly distributed among Middle East and North Africa doctors to assess their knowledge and practices on introduction of complementary feeding, impact of ID, its prevention, and their impression on prevalence of ID. Descriptive statistics were used. RESULTS: We received 2444 completed questionnaires. Thirty-nine percent of physicians do not follow the European Society for Paediatric Gastroenterology, Hepatology and Nutrition guidelines regarding age of introduction of complementary feedings. Approximately 62% estimate the prevalence of ID anemia to be 40% to 70%; however, only 17% always monitor hemoglobin between 9 and 12 months of age, 43% do so "almost" always, whereas 36% do so "rarely" or (4%) "never." For the prevention of ID in infants older than 6 months of age, almost all recommend introducing Fe supplements. Ninety-seven percent agree that untreated ID during infancy may have long-term negative effects on cognitive function, whereas 53.26% consider that Fe-enriched infant cereals result in staining of the baby teeth, constipation, and dark stools. CONCLUSIONS: Although there is awareness of the impact of ID, there are some misconceptions regarding age of introduction of complementary feedings, surveillance of Fe status, and side effects of Fe-enriched infant cereals. There is a need for educational initiatives focusing on prevention of Fe deficiency.
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Anemia Ferropénica/prevención & control , Conducta Alimentaria/psicología , Conocimientos, Actitudes y Práctica en Salud , Fenómenos Fisiológicos Nutricionales del Lactante , Médicos/psicología , África del Norte , Anemia Ferropénica/psicología , Suplementos Dietéticos , Femenino , Humanos , Lactante , Hierro/sangre , Deficiencias de Hierro , Masculino , Medio OrienteRESUMEN
BACKGROUND: Regional evidence-based guidelines for the prophylaxis and management of infantile colic are not available for the Middle East and North Africa (MENA) region. The Allied Against Infantile Functional GI Disorders (ACT) Working Group was created in January, 2015 to determine the knowledge gaps and the current management practices of infantile colic by physicians in the MENA region. The ACT group determined the need for a survey to address these questions. The objectives of the survey were to highlight current clinical practices on the management of infantile colic and to raise awareness on colic severity in the MENA region. METHODS: The ACT working group developed the survey which included respondent characteristics and closed questions on practice in colic prevention. The survey was subject to validation and ethics committee approval in all countries. RESULTS: A total of 1628 physicians (mostly pediatricians (75.4%), neonatologists (2.4%) and general practitioners (19.8%)) responded to the survey. The 5 most represented countries were KSA (27.9%), Kuwait (22.1%), Morocco (13.8%), Lebanon (10.6%), and Iraq (7.4%). Most of the respondents (77.8%) practiced in governmental settings. A majority of respondents (91.7%) reported that colic is diagnosed predominantly by clinical examination. Above 63%, of pediatricians surveyed, believed that the colic prevalence rate was >40%, which is greater than the 20% rate reported in worldwide surveys. Yet, most of the responding physicians (73%) prefer to simply reassure parents rather than prescribe a therapeutic agent. Most physicians were either neutral (58%) or did not endorse (18.4%) colic prophylaxis. Of those who prescribed formulae for non-breastfed children, a majority (64.3%) chose "Comfort" formulae over hydrolyzed or lactose-free formulae or formulae with probiotics. CONCLUSIONS: The results of this survey suggest that a substantial proportion of responding physicians from the selected MENA countries do not advocate for prophylaxis of colic. The findings of this survey suggest that more educational efforts are required to increase awareness of the strong body of evidence supporting the efficacy of probiotics in the prevention and management of infantile colic.
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Actitud del Personal de Salud , Competencia Clínica/estadística & datos numéricos , Cólico/terapia , Enfermedades Gastrointestinales/terapia , Pediatras/estadística & datos numéricos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Adulto , África del Norte/epidemiología , Cólico/diagnóstico , Cólico/epidemiología , Femenino , Enfermedades Gastrointestinales/diagnóstico , Enfermedades Gastrointestinales/epidemiología , Encuestas de Atención de la Salud , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Medio Oriente/epidemiologíaRESUMEN
BACKGROUND: Childhood obesity is one of the most serious public health issues of the twenty-first century affecting even low- and middle-income countries. Overweight and obese children are more likely to stay obese into adulthood. Due to the paucity of data on local practices, our study aimed to assess the knowledge and practices of physicians from the Middle East and North Africa region with respect to early-onset obesity. METHODS: A specific questionnaire investigating the perception and knowledge on early-onset obesity was circulated to healthcare providers (general physicians, pediatricians, pediatric gastroenterologist, neonatologists) practicing in 17 Middle East and North African countries. RESULTS: A total of 999/1051 completed forms (95% response) were evaluated. Of all respondents, 28.9% did not consistently use growth charts to monitor growth during every visit and only 25.2% and 46.6% of respondents were aware of the correct cut-off criterion for overweight and obesity, respectively. Of those surveyed, 22.3, 14.0, 36.1, 48.2, and 49.1% of respondents did not consider hypertension, type 2 diabetes, coronary heart disease, fatty liver disease, and decreased life span, respectively, to be a long-term complication of early childhood obesity. Furthermore, only 0.7% of respondents correctly answered all survey questions pertaining to knowledge of early childhood overweight and obesity. CONCLUSION: The survey highlights the low use of growth charts in the evaluation of early childhood growth in Middle East and North Africa region, and demonstrated poor knowledge of healthcare providers on the short- and long-term complications of early-onset obesity. This suggests a need for both continued professional education and development, and implementation of guidelines for the prevention and management of early childhood overweight and obesity.
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Competencia Clínica/estadística & datos numéricos , Obesidad Infantil , Pautas de la Práctica en Medicina/estadística & datos numéricos , Adulto , África del Norte , Preescolar , Femenino , Gráficos de Crecimiento , Encuestas de Atención de la Salud , Humanos , Lactante , Masculino , Persona de Mediana Edad , Medio Oriente , Obesidad Infantil/complicaciones , Obesidad Infantil/diagnóstico , Obesidad Infantil/terapiaRESUMEN
BACKGROUND: A number of scientific organisations have developed guidelines for the primary prevention of allergic disease through nutritional interventions. However, even if the best evidence-based guidelines are available, these guidelines do not necessarily lead to adherence and improved health outcomes. METHOD: To determine how closely the practice of physicians in select Middle Eastern and North African countries compares with the current recommendations on the primary prevention of allergy a survey study was performed using a structured questionnaire and convenience sampling. RESULTS: A total of 1481 physicians responded, of which 66.1% were pediatricians. A total of 76.6% of responding physicians routinely identify infants who are at risk for developing allergy. In infants at risk for developing allergy, 89.1% recommend exclusive breastfeeding for at least 4 months. In contrast to current recommendations, 51.6% routinely recommend avoidance of any allergenic food in the lactating mother. In infants at risk of developing allergy who are completely formula fed, standard infant formula was recommended by 22.5% of responders. Of the responding physicians, 50.6% would recommend delaying the introduction of complementary food in infants at risk of allergy compared to those not at risk, whereas 62.5% would recommend postponing the introduction of potentially allergenic foods. Only 6.6% stated they follow all current recommendations on food allergy prevention. CONCLUSION: The results of this survey suggest that a substantial part of responding physicians from select Middle Eastern and North African (MENA) countries do not follow current recommendations on primary prevention of allergic disease through nutritional interventions.
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Hipersensibilidad a los Alimentos/prevención & control , Adhesión a Directriz/estadística & datos numéricos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Prevención Primaria/métodos , Adulto , África del Norte , Lactancia Materna , Femenino , Encuestas de Atención de la Salud , Humanos , Lactante , Fórmulas Infantiles , Fenómenos Fisiológicos Nutricionales del Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Medio Oriente , Guías de Práctica Clínica como Asunto , Prevención Primaria/normasRESUMEN
Eosinophilic esophagitis (EoE) is a chronic, progressive, type 2 inflammatory esophageal disease presenting as dysphagia to solid food and non-obstructive food impaction. Knowledge gaps exist in its diagnosis and management. These expert recommendations focused on the diagnosis of EoE in the United Arab Emirates. An electronic search of PubMed and Embase databases was used to gather evidence from systematic reviews, randomized controlled trials, consensus papers, and expert opinions from the last five years on the diagnosis of EoE. The evidence was graded using the Oxford system. Literature search findings were shared with the expert panel. A 5-point scale (strongly agree, agree, neither agree nor disagree, disagree, and strongly disagree) was used, and a concordance rate of >75% among experts indicated agreement. Using a modified Delphi technique, 18 qualified experts provided 17 recommendations. Eleven statements achieved high agreement, four got moderate agreement, and two got low agreement. Challenges exist in diagnosing EoE, particularly in children. Esophageal biopsies were crucial in diagnosis, irrespective of visible mucosal changes. Further research on diagnostic tools like endoscopic mucosal impedance and biomarkers is needed. Diagnosis relies on esophageal biopsies and symptom-histology correlation; however, tools like EoE assessment questionnaires and endoscopic mucosal impedance could enhance the accuracy and efficiency of EoE diagnosis. The diagnosis of EoE is challenging since the symptoms seldom correlate with the histological findings. Currently, diagnosis is based on patient symptoms and endoscopic and histological findings. Further research into mucosal impedance tests and the role of biomarkers is needed to facilitate diagnosis.
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When oral feeding cannot provide adequate nutritional support to children, enteral tube feeding becomes a necessity. The overall aim is to ultimately promote appropriate growth, improve the patient's quality of life and increase carer satisfaction. Nasogastric tube feeding is considered appropriate on a short-term basis. Alternatively, gastrostomy feeding offers a more convenient and safer feeding option especially as it does not require frequent replacements, and carries a lower risk of complications. Gastrostomy tube feeding should be considered when nasogastric tube feeding is required for more than 2-3 wk as per the ESPEN guidelines on artificial enteral nutrition. Several techniques can be used to insert gastrostomies in children including endoscopic, image guided and surgical gastrostomy insertion whether open or laparoscopic. Each technique has its own advantages and disadvantages. The timing of gastrostomy insertion, device choice and method of insertion is dependent on the local expertise, patient requirements and family preference, and should be individualized with a multidisciplinary team approach. We aim to review gastrostomy insertion in children including indications, contraindications, history of gastrostomy, insertion techniques and complications.
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Coronavirus disease 2019 (COVID-19) typically presents with fever and respiratory symptoms in children. Most children develop an asymptomatic and mild illness, with a minority requiring specialist medical care. Gastrointestinal manifestations and liver injury can also occur in children following infection. The mechanisms of liver injury may include infection following direct viral hepatic tissue invasion, immune response, or medication effects. Affected children might develop mild liver dysfunction which has a benign course in most children with no pre-existing liver disease. However, the presence of non-alcoholic fatty liver disease or other pre-existing chronic liver disorders is associated with a higher risk of developing severe COVID-19 illness with poor outcomes. On the other hand, the presence of liver manifestations is associated with the severity of COVID-19 disease and is considered an independent prognostic factor. Respiratory, hemodynamic, and nutritional supportive therapies are the mainstay of management. Vaccination of children at increased risk of developing severe COVID-19 disease is indicated. This review describes the liver manifestations in children with COVID-19, detailing its epidemiology, basic mechanisms, clinical expression, management, and prognosis in those with and without pre-existing liver disease and also children who have had earlier liver transplantation.
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Objective: This study estimates the budgetary impact of the introduction of amino-acid formula (AAF) as first-line management in the elimination diet of infants with suspected cow milk protein allergy (CMPA) in the Kingdom of Saudi Arabia (KSA), Kuwait, and the United Arab Emirates (UAE) from a health-care payer's perspective. Methods: A global decision tree model was adapted to compare estimated costs in current practice (extensively hydrolyzed formula [eHF] or soy formula [SF] with the proposed approach of early introduction of AAF as first-line treatment of CMPA in non-breast-fed infants). Model inputs were derived from explorative literature reviews and medical experts' opinions. All costs were reported in local currency, ie, Saudi Riyal (SAR) for KSA, Kuwaiti Dinar (KWD) for Kuwait, and United Arab Emirates Dirham (AED) for the UAE. Results: Cost savings with the early introduction of AAF were 10% (SAR 15102542) in KSA, 10% (KWD 306565) in Kuwait, 17% (AED 1842018) in the UAE government sector and 13% (AED 4232932) in the UAE private sector. The highest cost reduction was observed in the cost of soy formula (SF), with a 58% reduction both in KSA (SAR 4204540) and UAE public sector (AED 110331). A significant cost reduction in medication costs in Kuwait (37%; KWD 5630) and medical examination costs in the UAE private sector (50%; AED 1508918) was observed. Conclusion: Results indicated that the introduction of AAF as the first line in the management of CMPA is a cost-saving strategy for the Gulf Cooperation Council (GCC) countries-KSA, Kuwait, and UAE- from a health-care payer's perspective.
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BACKGROUND: Despite its high incidence among children under the age of five, little is known about the burden of pediatric gastroenteritis outside the medical setting. The objective of this study was to describe the burden of acute gastroenteritis among children residing in the United Arab Emirates, including those not receiving medical care. METHODS: A quantitative cross-sectional survey of 500 parents of children under 5 years of age who had suffered from acute gastroenteritis the preceding three months was conducted in the cities of Abu Dhabi and Al Ain. Data collected included respondent characteristics, disease symptoms, medical care sought, and parental expenditures and work loss. Data were analyzed using parametric and non-parametric statistical methods. RESULTS: Vomiting and diarrhea episodes lasted on average between 3 and 4 days. Overall, 87% of parents sought medical care for their children; 10% of these cases required hospitalization with an average length of stay of 2.6 days. When medical care was sought, the average parental cost per gastroenteritis episode was US$64, 4.5 times higher than with home care only (US$14). Nearly 60% of this difference was attributable to co-payments and medication use: 69% of children used oral rehydration solution, 68% antiemetics, 65% antibiotics and 64% antidiarrheals. Overall, 38 parents missed work per 100 gastroenteritis episodes for an average of 1.4 days. CONCLUSIONS: Given its high incidence, pediatric gastroenteritis has an important financial and productivity impact on parents in the United Arab Emirates. To reduce this impact, efforts should be made both to prevent acute gastroenteritis and to optimize its treatment.
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Costo de Enfermedad , Gastroenteritis/economía , Enfermedad Aguda , Preescolar , Estudios Transversales , Diarrea/economía , Diarrea/etiología , Diarrea/terapia , Gastroenteritis/complicaciones , Gastroenteritis/diagnóstico , Gastroenteritis/terapia , Gastos en Salud/estadística & datos numéricos , Encuestas Epidemiológicas , Hospitalización/economía , Hospitalización/estadística & datos numéricos , Humanos , Lactante , Recién Nacido , Permiso Parental/estadística & datos numéricos , Emiratos Árabes Unidos , Vómitos/economía , Vómitos/etiología , Vómitos/terapiaRESUMEN
Objective: The majority of pediatric severe acute respiratory syndrome coronavirus 2 (COVID-19) cases demonstrate asymptomatic, mild or moderate disease. The main symptoms in children with COVID-19 are respiratory symptoms but some patients develop gastrointestinal symptoms and liver injury. We aim to review gastrointestinal symptoms and liver injury in children with confirmed COVID-19 infection. Method: This is a retrospective case note review of children with positive COVID-19 nasal Polymerase Chain Reaction aged 0-18 years admitted to a tertiary pediatric hospital from March 1st till June 1st 2020. Results: 180 children were identified. Mean age was 5 years (Range: 0.01-17), the majority of patients were school aged (30%). Patients were mainly from East Asia 81 (45%) and Arabs 67 (37%). Gastrointestinal symptoms were encountered in 48 (27%) patients and 8 (4%) patients had only Gastrointestinal symptoms with no associated fever or respiratory symptoms. Liver injury was seen in 57 (32%) patients. Patients with fever and cough were more likely to have gastrointestinal symptoms (P = <0.001 and 0.004 respectively). Fever was more likely to be associated with liver injury (P = 0.021). Children with abdominal pain were more likely to have elevated C-Reactive Protein (P = 0.037). Patients with diarrhea and vomiting were more likely to have elevated procalcitonin (P = 0.034 and 0.002 respectively). Children with Gastrointestinal symptoms were not more likely to be admitted to Pediatric Intensive Care Unit (P = 0.57). Conclusion: COVID-19 infection in children can display gastrointestinal symptoms at initial presentation. Additionally, gastrointestinal symptoms can be the only symptoms patients display. We demonstrated that children with gastrointestinal symptoms and liver injury can develop more severe COVID-19 disease and are more likely to have fever, cough, and raised inflammatory markers. Identifying children with gastrointestinal manifestations needs to be part of the initial screening assessment of children.What is known?⢠Pediatric COVID-19 cases mostly demonstrate asymptomatic, mild or moderate disease.⢠The symptoms in children are mainly respiratory but some display gastrointestinal symptoms.⢠Children with COVID-19 display increased gastrointestinal symptoms when compared to adults.What is new?⢠Children with COVID-19 displaying gastrointestinal symptoms are more likely to have fever, cough and elevated inflammatory markers.⢠Children with liver injury are more likely to develop fever.⢠Children with gastrointestinal involvement in COVID-19 are more likely to demonstrate more severe disease but are not more likely to be admitted to PICU.
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Objectives: Advancements in pediatric percutaneous endoscopic gastrostomy placement (PEG), laparoscopic-assisted gastrostomy (LAG) technique, and laparoscopic-assisted percutaneous endoscopic gastrostomy (LAPEG) procedure have opened up new options for gastrostomy tube placement. LAPEG utilizes endoscopy and laparoscopy for gastrostomy insertion. This review compares the outcomes and complications of LAG and LAPEG techniques in children. Methods: All LAG and LAPEG gastrostomy tube placements in children from September 2010 to September 2019 were reviewed retrospectively. Patient demographic, along with procedural and 1-year complication data, were collected. Results: In total, 92/181 of gastrostomies were LAG and 89/181 were LAPEG. The mean age, weight and patient characteristics were comparable. Conversion rate was 1% in both groups (p = 0.74), there was no peritoneal leak in either group, a minor serosal injury to the stomach was seen in 1 patient in LAG with no bowel injury in LAPEG cohort (p = 0.51), need for re-operation was 1 and 2% in LAG and LAPEG, respectively (p = 0.49), early tube dislodgement was in 8 (9%) patients in LAG and 7 (6%) in LAPEG (p = 0.53) and wound infection was 13/92 in LAG and 11/89 in LAPEG (p = 0.8). The median operative time for LAPEG was less than LAG (p < 0.001) by 11 min but the median length of hospital stay was not significantly different (p < 0.096). Conclusion: Both LAG and LAPEG techniques in children are safe with comparable complication rates and length of hospital stay, the addition of endoscopy to LAG allowed for shorter operative time in the LAPEG technique.
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Functional gastrointestinal disorders (FGIDs) refer to gastrointestinal tract issues that lack clear structural or biochemical causes. Their pathophysiology is still unclear, but gut microbiota alterations are thought to play an important role. This systematic review aimed to provide a comprehensive overview of the faecal microbiota of infants and young children with FGIDs compared to healthy controls. A systematic search and screening of the literature resulted in the inclusion of thirteen full texts. Most papers reported on infantile colic, only one studied functional constipation. Despite methodological limitations, data show alterations in microbial diversity, stability, and colonisation patterns in colicky infants compared to healthy controls. Several studies (eight) reported increases in species of (pathogenic) Proteobacteria, and some studies (six) reported a decrease in (beneficial) bacteria such as Lactobacilli and Bifidobacteria. In addition, accumulation of related metabolites, as well as low-grade inflammation, might play a role in the pathophysiology of infantile colic. Infants and toddlers with functional constipation had significantly lower levels of Lactobacilli in their stools compared to controls. Microbial dysbiosis and related changes in metabolites may be inherent to FGIDs. There is a need for more standardised methods within research of faecal microbiota in FGIDs to obtain a more comprehensive picture and understanding of infant and childhood FGIDs.
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Enfermedades Gastrointestinales , Microbioma Gastrointestinal , Microbiota , Niño , Preescolar , Disbiosis/complicaciones , Heces , Enfermedades Gastrointestinales/etiología , Humanos , LactanteRESUMEN
ABSTRACT: Exclusive breast feeding is recommended in all guidelines as the first choice feeding. Cow milk allergy (CMA) can be diagnosed by a diagnostic elimination diet for 2 to 4 weeks with a hypo-allergenic formula, followed by a challenge test with intact cow milk protein. The most often used hypo-allergenic formula for the diagnostic elimination diet and the therapeutic diet is a CM based extensive hydrolysate. CM-based partial hydrolysates cannot be recommended in the management of CMA because of insufficient efficacy and possible reactions, but about half of the infants with CMA may tolerate a partial hydrolysate. The pros and cons of other dietary options are discussed in this paper. The use of an amino acid-based formula and/or rice based hydrolysate formula during the diagnostic elimination and therapeutic diet is debated. When available, there is sufficient evidence to consider rice hydrolysates as an adequate alternative to CM-based hydrolysates, since some infants will still react to the CM hydrolysate. The pros and cons of dietary options such as soy formula, buckwheat, almond, pea or other plant based dietary products are discussed. Although the majority of the plant-based beverages are nutritionally inadequate, some are nutritionally adapted for toddlers. However, accessibility and content vary by country and, thus far there is insufficient evidence on the efficacy and tolerance of these plant-based drinks (except for soy formula and rice hydrolysates) to provide an opinion on them. CONCLUSION: A diagnostic elimination diet, followed by a challenge remains the diagnostic standard. The use of an awareness tool may result in a decrease of delayed diagnosis. Breastmilk remains the ideal source of nutrition and when not available a CM extensively hydrolyzed formula, rice hydrolysate or amino acid formula should be recommended. More evidence is needed regarding plant-based drinks.
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Myosin Vb (MYO5B) is a motor protein that facilitates protein trafficking and recycling in polarized cells by RAB11- and RAB8-dependent mechanisms. Biallelic MYO5B mutations are identified in the majority of patients with microvillus inclusion disease (MVID). MVID is an intractable diarrhea of infantile onset with characteristic histopathologic findings that requires life-long parenteral nutrition or intestinal transplantation. A large number of such patients eventually develop cholestatic liver disease. Bi-allelic MYO5B mutations are also identified in a subset of patients with predominant early-onset cholestatic liver disease. We present here the compilation of 114 patients with disease-causing MYO5B genotypes, including 44 novel patients as well as 35 novel MYO5B mutations, and an analysis of MYO5B mutations with regard to functional consequences. Our data support the concept that (1) a complete lack of MYO5B protein or early MYO5B truncation causes predominant intestinal disease (MYO5B-MVID), (2) the expression of full-length mutant MYO5B proteins with residual function causes predominant cholestatic liver disease (MYO5B-PFIC), and (3) the expression of mutant MYO5B proteins without residual function causes both intestinal and hepatic disease (MYO5B-MIXED). Genotype-phenotype data are deposited in the existing open MYO5B database in order to improve disease diagnosis, prognosis, and genetic counseling.
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The latest definition of a prebiotic is "a substrate that is selectively utilized by host microorganisms conferring a health benefit"; it now includes non-food elements and is applicable to extra-intestinal tissues. Prebiotics are recognized as a promising tool in the promotion of general health and in the prevention and treatment of numerous juvenile diseases. Prebiotics are considered an immunoactive agent, with the potential for long-lasting effects extending past active administration of the prebiotic. Because of its extremely low risk of serious adverse effects, ease of administration, and strong potential for influencing the composition and function of the microbiota in the gut and beyond, the beneficial clinical applications of prebiotics are expanding. Prebiotics are the third largest component of human breast milk. Preparations including galactooligosaccharides (GOS), fructooligosaccharides (FOS), 2'-fucosyllactose, lacto-N-neo-tetraose are examples of commonly used and studied products for supplementation in baby formula. In particular, the GOS/FOS combination is the most studied. Maintaining a healthy microbiome is essential to promote homeostasis of the gut and other organs. With more than 1,000 different microbial species in the gut, it is likely more feasible to modify the gut microbiota through the use of certain prebiotic mixtures rather than supplementing with a particular probiotic strain. In this review, we discuss the latest clinical evidence regarding prebiotics and its role in gut immunity, allergy, infections, inflammation, and functional gastrointestinal disorders.
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OBJECTIVES: Guidance and evidence supporting routine use of partially hydrolyzed formula (pHF) versus intact cows' milk protein (CMP) formula are limited in non-exclusively breastfed infants. The aim of this review was to better clarify issues of routine use of pHF in non-exclusively breastfed infants who are not at risk for allergic disease by using a systematic review and Delphi Panel consensus. METHODS: A systematic review and Delphi consensus panel (consisting of eight8 international pediatric allergists and gastroenterologists) was conducted to evaluate evidence supporting growth, tolerability, and effectiveness of pHF in non-exclusively breastfed infants. RESULTS: None of the studies reviewed identified potential harm of pHF use compared with CMP in non-exclusively breastfed infants. There was an expert consensus that pHF use is likely as safe as intact CMP formula, given studies suggesting these have comparable nutritional parameters. No high-quality studies were identified evaluating the use of pHF to prevent allergic disease in non-exclusively breastfed infants who are not at risk for allergic disease (e.g., lacking a parental history of allergy). Limited data suggest that pHF use in non-exclusively breastfed infants may be associated with improved gastric emptying, decreased colic incidence, and other common functional gastrointestinal symptoms compared with CMP. However, because the data are of insufficient quality, the findings from these studies have to be taken with caution. No studies were identified that directly compared the different types of pHF, but there was an expert consensus that growth, allergenicity, tolerability, effectiveness, and clinical role among such pHF products may differ. CONCLUSIONS: Limited data exist evaluating routine use of pHFs in non-exclusively breastfed infants, with no contraindications identified in the systematic review. An expert consensus considers pHFs for which data were available to be as safe as CMP formula as growth is normal. The preventive effect on allergy of pHF in infants who are not at risk for allergic disease has been poorly studied. Cost of pHF versus starter formula with intact protein differs from country to country. However, further studies in larger populations are needed to clinically confirm the benefits of routine use of pHF in non-exclusively breastfed infants. These studies should also address potential consumer preference bias.
Asunto(s)
Fórmulas Infantiles/química , Fenómenos Fisiológicos Nutricionales del Lactante/efectos de los fármacos , Proteínas de la Leche/farmacología , Hidrolisados de Proteína/farmacología , Animales , Lactancia Materna , Bovinos , Consenso , Humanos , Hidrólisis , Lactante , Fórmulas Infantiles/efectos adversos , Leche , Proteínas de la Leche/efectos adversos , Hidrolisados de Proteína/efectos adversosRESUMEN
OBJECTIVES: To estimate the cost of functional gastrointestinal disorders (FGIDs) and related signs and symptoms in infants to the third party payer and to parents. STUDY DESIGN: To estimate the cost of illness (COI) of infant FGIDs, a two-stage process was applied: a systematic literature review and a COI calculation. As no pertinent papers were found in the systematic literature review, a 'de novo' analysis was performed. For the latter, the potential costs for the third party payer (the National Health Service (NHS) in England) and for parents/carers for the treatment of FGIDs in infants were calculated, by using publicly available data. In constructing the calculation, estimates and assumptions (where necessary) were chosen to provide a lower bound (minimum) of the potential overall cost. In doing so, the interpretation of the calculation is that the true COI can be no lower than that estimated. RESULTS: Our calculation estimated that the total costs of treating FGIDs in infants in England were at least £72.3 million per year in 2014/2015 of which £49.1 million was NHS expenditure on prescriptions, community care and hospital treatment. Parents incurred £23.2 million in costs through purchase of over the counter remedies. CONCLUSIONS: The total cost presented here is likely to be a significant underestimate as only lower bound estimates were used where applicable, and for example, costs of alternative therapies, inpatient treatments or diagnostic tests, and time off work by parents could not be adequately estimated and were omitted from the calculation. The number and kind of prescribed products and products sold over the counter to treat FGIDs suggest that there are gaps between treatment guidelines, which emphasise parental reassurance and nutritional advice, and their implementation.