ABSTRACT
Eosinophilic esophagitis is a chronic inflammatory disease characterized by esophageal dysfunction and progression to fibrosis. Its incidence is increasing in our setting with deep regional variations. To corroborate this hypothesis, a longitudinal, retrospective, multicenter observational study was carried out of patients who received a diagnosis of eosinophilic esophagitis from 2008 to 2022 at public hospitals in the province of Zaragoza. The annual incidence rates and mean incidence rate were calculated using the data for the reference population. A total of 104 patients were included. The mean incidence rate was 5.1 cases per 100,000 inhabitants < 15 years old/year (0.75-11.2). In the first five-year period (2008-2012) the rate was 1.2 cases per 100,000 inhabitants/year, compared with a rate of 6 cases per 100,000 inhabitants/year in the second 5-year period (2013-2017), [OR 5,68 (IC 95% 2,55 - 12,67, p < 0,05]; and 8.1 cases per 100,000 inhabitants/year in the third five-year period (2018-2022), [OR 7,74 (IC 95% 3,52 - 16,99, p < 0,05] It is concluded that eosinophilic esophagitis incidence has increased among the child population of Zaragoza over the past 15 years, with a 7-fold higher risk of having the condition in the third five-year period compared with the first one.
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INTRODUCTION: The prevalence of malnutrition among infants with congenital heart disease (CHD) is high. Early nutritional assessment and intervention contribute significantly to its treatment and improve outcomes. Our objective was to develop a consensus document for the nutritional assessment and management of infants with CHD. MATERIAL AND METHODS: We employed a modified Delphi technique. Based on the literature and clinical experience, a scientific committee prepared a list of statements that addressed the referral to paediatric nutrition units (PNUs), assessment, and nutritional management of infants with CHD. Specialists in paediatric cardiology and paediatric gastroenterology and nutrition evaluated the questionnaire in 2 rounds. RESULTS: Thirty-two specialists participated. After two evaluation rounds, a consensus was reached for 150 out of 185 items (81%). Cardiac pathologies associated with a low and high nutritional risk and associated cardiac or extracardiac factors that carry a high nutritional risk were identified. The committee developed recommendations for assessment and follow-up by nutrition units and for the calculation of nutritional requirements, the type of nutrition and the route of administration. Particular attention was devoted to the need for intensive nutrition therapy in the preoperative period, the follow-up by the PNU during the postoperative period of patients who required preoperative nutritional care, and reassessment by the cardiologist in the case nutrition goals are not achieved. CONCLUSIONS: These recommendations can be helpful for the early detection and referral of vulnerable patients, their evaluation and nutritional management and improving the prognosis of their CHD.
Subject(s)
Heart Defects, Congenital , Malnutrition , Infant , Child , Humans , Consensus , Nutritional Status , Nutritional Support , Malnutrition/diagnosis , Heart Defects, Congenital/complications , Heart Defects, Congenital/therapy , Heart Defects, Congenital/diagnosisABSTRACT
Introducción: La tasa de desnutrición entre los lactantes con cardiopatías congénitas (CC) es elevada. Una evaluación e intervención nutricional tempranas ayudan a su tratamiento y mejoran el pronóstico. El objetivo fue elaborar un documento de consenso para la evaluación y el tratamiento nutricional del lactante con CC. Material y métodos: Se utilizó una técnica Delphi modificada. Con base en la literatura y en su experiencia clínica, un comité científico elaboró un listado de afirmaciones que abordaban la derivación a unidades de nutrición pediátrica (UNP), la evaluación y el manejo nutricional de los lactantes con CC. Especialistas en cardiología pediátrica, y gastroenterología y nutrición pediátrica evaluaron el cuestionario en dos rondas. Resultados: Participaron 32 especialistas. Tras dos rondas de evaluación, se consensuaron 150 de 185 ítems (81%). Se determinaron patologías cardiacas de bajo y alto riesgo nutricional y factores asociados cardiacos o extracardiacos que confieren riesgo nutricional alto. Se elaboraron recomendaciones para la evaluación y seguimiento en unidades de nutrición y sobre el cálculo de los requerimientos nutricionales, el tipo de nutrición y la vía de administración. Se enfatiza la necesidad de un tratamiento nutricional intensivo en el preoperatorio, del seguimiento por la UNP en el postoperatorio cuando se haya necesitado intervención preoperatoria, y de la reevaluación por el cardiólogo cuando no se alcancen los objetivos nutricionales. Conclusiones: Estas recomendaciones pueden ser de ayuda para la detección precoz y derivación temprana de población vulnerable, su evaluación y tratamiento nutricional y para mejorar el pronóstico de su CC. (AU)
Introduction: The prevalence of malnutrition among infants with congenital heart disease (CHD) is high. Early nutritional assessment and intervention contribute significantly to its treatment and improve outcomes. Our objective was to develop a consensus document for the nutritional assessment and management of infants with CHD. Material and methods: We employed a modified Delphi technique. Based on the literature and clinical experience, a scientific committee prepared a list of statements that addressed the referral to paediatric nutrition units (PNUs), assessment, and nutritional management of infants with CHD. Specialists in paediatric cardiology and paediatric gastroenterology and nutrition evaluated the questionnaire in 2 rounds. Results: Thirty-two specialists participated. After two evaluation rounds, a consensus was reached for 150 out of 185 items (81%). Cardiac pathologies associated with a low and high nutritional risk and associated cardiac or extracardiac factors that carry a high nutritional risk were identified. The committee developed recommendations for assessment and follow-up by nutrition units and for the calculation of nutritional requirements, the type of nutrition and the route of administration. Particular attention was devoted to the need for intensive nutrition therapy in the preoperative period, the follow-up by the PNU during the postoperative period of patients who required preoperative nutritional care, and reassessment by the cardiologist in the case nutrition goals are not achieved. Conclusions: These recommendations can be helpful for the early detection and referral of vulnerable patients, their evaluation and nutritional management and improving the prognosis of their CHD. (AU)
Subject(s)
Humans , Male , Female , Infant , Nutritional Support , Nutrition Therapy , Heart Defects, Congenital , Nutrition Assessment , Malnutrition , Nutrition Disorders , ConsensusABSTRACT
Hypertransaminasemia is a frequent finding in pediatrics, which could reflect potentially treatable serious disease. The aim of this document is to establish, by reviewing the available evidence, a consensus for an adequate management of hypertransaminasemia, from its detection until the study is complete. To this end, a working group was formed with the participation of members of the Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the Spanish Association of Primary Care Pediatrics (AEPap) and the Spanish Society of Primary Care Pediatrics (SEPEAP). Twenty-one recommendations are established with a marked practical component that will be useful in hospital clinical practice and primary care.
Subject(s)
Pediatrics , Child , Consensus , HumansABSTRACT
La hipertransaminasemia es un hallazgo frecuente en pediatría, puede ser banal o reflejar enfermedad grave potencialmente tratable. El objetivo de este documento es establecer, mediante la revisión de la evidencia disponible, un consenso para un adecuado enfoque práctico desde la detección de la hipertransaminasemia hasta completar su estudio en la edad pediátrica. Para ello, se constituyó un grupo de trabajo con participación de miembros de la Sociedad de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP), Asociación Española de Pediatría de Atención Primaria (AEPap) y Sociedad Española de Pediatría de Atención Primaria (SEPEAP). Se establecieron 21 recomendaciones con el objetivo de que sirvan de utilidad en la práctica clínica habitual tanto en atención primaria como hospitalaria. (AU)
Hypertransaminasemia is a frequent finding in pediatrics, which could reflect potentially treatable serious disease. The aim of this document is to establish, by reviewing the available evidence, a consensus for an adequate management of hypertransaminasemia, from its detection until the study is complete. To this end, a working group was formed with the participation of members of the Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the Spanish Association of Primary Care Pediatrics (AEPap) and the Spanish Society of Primary Care Pediatrics (SEPEAP). Twenty-one recommendations are established with a marked practical component that will be useful in hospital clinical practice and primary care. (AU)
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Catastrophic Illness , Pediatrics , Physicians, Primary Care , Non-alcoholic Fatty Liver Disease , TransaminasesSubject(s)
Humans , Male , Child , Cholestasis, Intrahepatic/diagnosis , Cholestasis, Intrahepatic/etiology , Cholestasis, Intrahepatic/drug therapy , Pruritus , Jaundice , Inpatients , Physical Examination , Symptom Assessment , Gastroenterology , Gastrointestinal Diseases , Pediatrics , Treatment OutcomeABSTRACT
INTRODUCTION: Although changes in liver function tests can be non-specific in numerous clinical conditions, they can be the first sign of a potentially serious disease in an asymptomatic patient. MATERIAL AND METHODS: Retrospective cohort study, performed by reviewing the records of children of a reference hospital central laboratory with alanine aminotransferase enzyme (ALT) elevation during a 6-month aleatory period. RESULTS: 572 blood tests with serum ALT elevation corresponding to 403 patients have been assessed during the period studied. 98 patients were excluded for presenting abnormal liver test before the study period of comorbidity that could produce ALT elevation. The remaining 305 patients, 22.6% were diagnosed with a medical condition during the first blood test that explained the ALT elevation, although only 33.3% of them were followed up until verifying their normalisation. Final study sample consists of 236 patients with abnormal liver test without apparent liver disease. Adequate follow-up was found only in 29% of them. From this group, 9 patients (13%) were diagnosed with liver disease. The rest of the samples were not properly monitored. In patients with higher serum ALT levels, follow-up was early and more appropriate. CONCLUSIONS: In our area, most children without apparent liver disease are no properly monitored. Therefore, an opportunity to diagnosis and treat a potential liver disease was lost in a great number of children. All children with unexplained hypertransaminasaemia must be studied.
Subject(s)
Liver Diseases , Alanine Transaminase , Child , Humans , Liver Diseases/diagnosis , Liver Function Tests , Retrospective StudiesABSTRACT
OBJECTIVE: to analyze the presence of common personality traits and anxiety states in children and adolescents with inflammatory bowel disease (IBD). PATIENTS AND METHOD: Longitudinal, prospecti ve, and analytical study by applying the questionnaires Children's Personality Questionnaire, High School Personality Questionnaire, State-Trait Anxiety Inventory for Children, and State-Trait Anxie ty Inventory for patients with IBD aged between 9 and 18 years seen at reference IBD units in Ara gon, Spain. The participants excluded were those with active disease, defined as a score > 10 on the Pediatric Crohn's Disease Activity Index (PCDAI Score) or > 10 on the Pediatric Ulcerative Colitis Activity Index (PUCAI Score). RESULTS: Twenty-six patients participated (73% male). 61.5% pre sented Crohn's disease (CD) and 38.5% ulcerative colitis (UC). No patient presented active disease. The personality profile as a group was characterized by being open, emotionally stable, calm, sober, sensible, enterprising, impressionable, dependent, serene, perfectionist, and relaxed. 50% of the CD patients were enterprising versus no UC patients (p < 0.05). There were no statistically significant di fferences when comparing the remaining personality factors based on IBD type, age, or sex. Patients with CD tended to be calmer (p = 0.0511) and patients with UC more introverted (p = 0.0549). The sample presented a state anxiety level (A/E) -1.1 ± 0.8 SD compared with the population average. The level of anxiety as a feature (A/R) was -0.6 ± 1 SD. Males had significantly lower levels than females in the case of A/E (p < 0.05). CONCLUSIONS: The presence of common personality traits in the pediatric population with IBD stands out but there was no greater anxiety than in the reference population.
Subject(s)
Anxiety/diagnosis , Colitis, Ulcerative/psychology , Crohn Disease/psychology , Personality Assessment , Adolescent , Child , Extraversion, Psychological , Female , Humans , Inflammatory Bowel Diseases/psychology , Introversion, Psychological , Male , Prospective Studies , Socioeconomic Factors , Spain , Surveys and QuestionnairesABSTRACT
Introducción: Las alteraciones del perfil hepático constituyen un hecho inespecífico propio de numerosas condiciones clínicas. Sin embargo, puede implicar la primera manifestación de una patología potencialmente grave en un paciente asintomático.Material y métodos: Estudio observacional retrospectivo que incluye todas las analíticas sanguíneas con elevación de alanino aminotransferasa (ALT) en pacientes pediátricos solicitadas en un sector sanitario en un período de 6meses.Resultados: Se registraron 572 analíticas correspondientes a 403 pacientes. Se excluyeron 98 pacientes con hipertransaminasemia ya conocida o comorbilidad. De los 305 restantes, el 22,6% se diagnosticaron de patología asociada a hipertransaminasemia, y de estos, se comprobó normalización en el 33,3%. De los 236 pacientes con hipertransaminasemia sin justificar se realizó un seguimiento en el 29%, encontrando patología hepática en 9pacientes (13% del grupo). En el resto de la muestra no se comprobó analíticamente la evolución de las transaminasas ni la presencia de posible patología hepática. Los pacientes con cifras más elevadas se controlan mejor y antes que los que presentan cifras más bajas.Conclusiones: En nuestra área, la mayoría de los niños sin enfermedad hepática aparente con hallazgo de ALT elevada no son adecuadamente controlados. Esto hace que se pierda una oportunidad única de diagnosticar y tratar precozmente una enfermedad hepática potencial en un gran número de niños. Todo niño con hipertransaminasemia inexplicada debe ser estudiado. (AU)
Introduction: Although changes in liver function tests can be non-specific in numerous clinical conditions, they can be the first sign of a potentially serious disease in an asymptomatic patient.Material and methods: Retrospective cohort study, performed by reviewing the records of children of a reference hospital central laboratory with alanine aminorransferase enzyme (ALT) elevation during a 6 month aleatory period.Results: 572 blood tests with serum ALT elevation corresponding to 403 patients had been assessed during the period studied. 98 patients were excluded for presenting abnormal liver test before the study period of comorbidity that could produce ALT elevation. The remaining 305 patients, 22.6% were diagnosed with a medical condition during the first blood test that explained the ALT elevation, although only 33.3% of them were followed up until verifying their normalization. Final study sample consists of 236 patients with abnormal liver test without apparent liver disease. Adequate follow-up was found only in 29% of them. From this group, 9 patients (13%) were diagnosed with liver disease. The rest of the sample were not properly monitored. In patients with higher serum ALT levels, follow-up was early and more appropiate.Conclusions: In our area, most children without apparent liver disease are no properly monitored. Therefore, an opportunity to diagnosis and treat a potential liver disease was lost in a great number of children. All children with unexplainedhypertransaminasaemia must be studied. (AU)
Subject(s)
Humans , Infant , Child, Preschool , Child , Adolescent , Liver , Liver Function Tests , Retrospective Studies , Epidemiology, DescriptiveABSTRACT
Vedolizumab is a humanised monoclonal antibody that binds to integrin α4ß7 expressed in T-cells, inhibiting its binding to the mucosal addressin cell adhesion molecule-1 (MAdCAM-1), which is specifically expressed in the small intestine and colon, playing a fundamental role in T-cell migration to the gastrointestinal tract. Vedolizumab has been shown to be effective in treating adults with inflammatory bowel disease; however, efficacy data for paediatric use are scarce. The objective of the present study was to assess the effectiveness and safety of vedolizumab for inducing and maintaining clinical remission in children with inflammatory bowel disease. We conducted a retrospective multicentre study of patients younger than 18 years with inflammatory bowel disease refractory to anti-tumour necrosis factor alpha (anti-TNF-α) drugs, who underwent treatment with vedolizumab. Clinical remission was defined as a score < 10 points in the activity indices. We included 42 patients, 22 of whom were male (52.3%), with a median age of 13.1 years (IQR 10.2-14.2) at the start of treatment. Of the 42 patients, 14 (33.3%) had Crohn's disease (CD) and 28 (66.7%) had ulcerative colitis (UC). At the start of treatment with vedolizumab, the Paediatric Crohn's Disease Activity Index was 36 (IQR 24-40) and the Paediatric Ulcerative Colitis Activity Index was 47 (IQR 25-65). All of them had received prior treatment with anti-TNF and 3 patients ustekinumab. At week 14, 69% of the patients responded to the treatment (57.1% of those with CD and 75% of those with UC; p=0.238), and 52.4% achieved remission (35.7% with CD and 60.7% with UC; p=0.126). At 30 weeks, the response rate was 66.7% (46.2% and 78.3% for CD and UC, respectively; p=0.049), and 52.8% achieved remission (30.8% and 65.2% for CD and UC, respectively; p=0.047). Among the patients with remission at week 14, 80% of the patients with CD and 84.5% of those with UC maintained the remission at 52 weeks. Adverse effects were uncommon and mild. Three patients (7.1%) presented headaches, 1 presented alopecia, 1 presented anaemia and 1 presented dermatitis.Conclusion: The results show that treatment with vedolizumab is a safe and effective option for achieving clinical remission in paediatric patients with inflammatory bowel disease with primary failure or loss of response to other treatments, especially in UC. What is Known: ⢠Vedolizumab is effective in inducing and maintaining remission in adult patients with inflammatory bowel disease. ⢠Most studies and clinical trials have been performed on adult populations, and there is currently no indication for paediatric populations. What is New: ⢠Children with inflammatory bowel disease refractory to anti-TNF presented higher clinical remission rates than those published for adults. ⢠There are few publications of this magnitude on paediatric populations treated with vedolizumab and with long-term follow-up (52 weeks).
Subject(s)
Colitis, Ulcerative , Inflammatory Bowel Diseases , Adolescent , Antibodies, Monoclonal, Humanized , Child , Colitis, Ulcerative/drug therapy , Female , Gastrointestinal Agents/adverse effects , Humans , Inflammatory Bowel Diseases/drug therapy , Male , Remission Induction , Retrospective Studies , Treatment Outcome , Tumor Necrosis Factor InhibitorsABSTRACT
La mucopolisacaridosis tipo III B es una enfermedad de depósito lisosomal causada por la deficiencia de la enzima N-acetil-alfa-d-glucosaminidasa, implicada en el catabolismo del heparán sulfato, que produce su acúmulo en diversos tejidos. Se presenta a un paciente de 8 años, afectado de mucopolisacaridosis tipo III B, con historia de diarrea crónica y hallazgos endoscópicos e histológicos compatibles con linfangiectasia intestinal. Tras tratamiento dietético con restricción de ácidos grasos de cadena larga y rica en triglicéridos de cadena media, presentó mejoría clínica, mantenida hasta la actualidad.La patogenia de la diarrea crónica en pacientes con mucopolisacaridosis tipo III B es aún desconocida. Debe investigarse la presencia de linfangiectasia intestinal en estos pacientes e iniciar, en caso de confirmarse, un tratamiento dietético adecuado para mejorar así su calidad de vida.
Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with medium-chain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.
Subject(s)
Humans , Male , Child , Lymphangiectasis, Intestinal/diagnostic imaging , Lysosomal Storage Diseases , Mucopolysaccharidosis III , Diet, Fat-Restricted , Diarrhea , Lymphangiectasis, Intestinal/therapyABSTRACT
Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The Linfangiectasia intestinal en un paciente afectado de síndrome de Sanfilippo B Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.
La mucopolisacaridosis tipo III B es una enfermedad de depósito lisosomal causada por la deficiencia de la enzima N-acetil-alfad- glucosaminidasa, implicada en el catabolismo del heparán sulfato, que produce su acúmulo en diversos tejidos. Se presenta a un paciente de 8 años, afectado de mucopolisacaridosis tipo III B, con historia de diarrea crónica y hallazgos endoscópicos e histológicos compatibles con linfangiectasia intestinal. Tras tratamiento dietético con restricción de ácidos grasos de cadena larga y rica en triglicéridos de cadena media, presentó mejoría clínica, mantenida hasta la actualidad. La patogenia de la diarrea crónica en pacientes con mucopolisacaridosis tipo III B es aún desconocida. Debe investigarse la presencia de linfangiectasia intestinal en estos pacientes e iniciar, en caso de confirmarse, un tratamiento dietético adecuado para mejorar así su calidad de vida.
Subject(s)
Mucopolysaccharidosis III , Acetylglucosaminidase , Child , Diarrhea/etiology , Heparitin Sulfate , Humans , Mucopolysaccharidosis III/complications , Mucopolysaccharidosis III/diagnosis , Quality of LifeSubject(s)
Abnormalities, Multiple/genetics , Esophageal Achalasia/genetics , Intracellular Signaling Peptides and Proteins/genetics , Abnormalities, Multiple/diagnostic imaging , Abnormalities, Multiple/physiopathology , Child , Esophageal Achalasia/diagnostic imaging , Esophageal Achalasia/physiopathology , Female , Humans , SyndromeABSTRACT
No disponible
Subject(s)
Humans , Male , Infant , Child , Dyslipidemias/etiology , Liver Diseases/etiology , Hepatomegaly/diagnostic imaging , Hypercholesterolemia/diagnosis , Sterol Esterase/deficiency , Wolman Disease/diagnosis , Wolman Disease/therapy , Dyslipidemias/diagnosis , Liver Diseases/diagnosis , Biopsy , Wolman Disease/genetics , Enzyme Replacement Therapy/methods , Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use , Liver/enzymology , Early DiagnosisABSTRACT
INTRODUCTION: Patients with moderate-severe cerebral palsy require the support of their caregivers to carry out the activities of daily living (ADLs). OBJECTIVES: To describe the comorbidities, need for care in children with cerebral palsy and to analyse the influence of the degree of motor involvement, nutritional status and other neurological disorders. METHODS: Cross-sectional and observational study. Patients with cerebral palsy degrees III-IV-V according to the Gross Motor Function Classification System (GMFCS) have been studied. A record of comorbidities has been made and body composition has been studied using anthropometry and bioimpedance. In addition, a caregiver burden survey on ADLs has been carried out (10 items on the different actions: hygiene, clothing, transfers, sleeping and feeding). Which variables have the greatest influence on the perception of difficulty in performing ADLs have been studied. RESULTS: A total of 69 patients (50.7% women, mean age 10.46 ± 0.4 years) were analysed, with GMFCS grades: grade III 36.2% (N=25), grade IV 29.0% (N=20), grade V 34.8% (N=24). A relationship was found between the caregiver burden score and GMFCS grade (P=0.003) and intellectual disability (P<0.001). However, regardless of the degree of GMFCS and intellectual disability, there is greater difficulty in performing ADLs in relation to lower values in weight (Z-score) (P=0.028), fat mass (kg) (P=0.035), fat mass (%) (P=0.094), body mass index (Z-score) (P=0.086). CONCLUSIONS: In addition to the degree of clinical impairment, nutritional status is a factor that influences the caregiver's difficulty in performing the ADLs in cerebral palsy patients on which we can act to improve this problem.
Subject(s)
Caregiver Burden , Cerebral Palsy , Nutritional Status , Activities of Daily Living , Cerebral Palsy/physiopathology , Cerebral Palsy/therapy , Child , Cross-Sectional Studies , Female , Humans , Male , Severity of Illness IndexSubject(s)
Dyslipidemias , Liver Diseases , Wolman Disease , Child , Dyslipidemias/etiology , Humans , Infant , Liver Diseases/etiology , Male , Wolman Disease/complications , Wolman Disease/diagnosis , Wolman DiseaseABSTRACT
Bone health problems may be related to the nutritional deficit in pediatric patients with cerebral palsy. It is common to find asymptomatic vertebral fractures when they have low bone mineral density. Fat mass deficit could be related to a lower bone mineral density and a higher risk of vertebral fractures. OBJECTIVES: To study the bone health of patients with CP and its relationship with neurological and nutritional status. PURPOSE: Cerebral palsy (CP) is the most common cause of motor disability in pediatric age. METHODS: Cross-sectional, observational, descriptive, and analytical study in which patients with CP between 4 and 5 years with Gross Motor Function Classification System (GMFCS) grades III-IV-V were included. It was carried out: survey, anthropometric study, bioimpedanciometry (BIA), and bone densitometry. Patients with low bone mineral density (BMD Z score less than - 2.0) underwent lumbar radiography looking for vertebral fractures to be diagnosed with osteoporosis. RESULTS: Total sample: 51 patients (51.0% women). Mean age: 11.0 ± 0.5 years. BMD Z score average: - 2.1 (95% CI - 2.5, - 1.7). BMD Z score according to GMFCS: grade III - 1.6 (- 2.2; - 1.), grade IV - 1.6 (- 2.4; - 0.9), grade V - 3.1 (- 3.9, - 2.2) (p = 0.013). Bone health classification according to the International Society for Clinical Densitometry was: 47.1% normal, 52.9% low BMD. Relationship between low BMD and low fat mass (p = 0.030) and low cell mass (p = 0.040) was found. Prevalence of vertebral fractures in lumbar radiography: 25.9%, increasing as the degree of neurological involvement. Vertebral fractures were found in 5/13 GMFCS grade V, 2/6 GMFCS grade IV, and 0/10 GMFCS grade III. CONCLUSIONS: Bone health in the pediatric population with CP is compromised in relation to the degree of neurological involvement and nutritional status. Those patients with moderate-severe cerebral palsy and low BMD seem to present an increased risk of fracture.
Subject(s)
Bone Density/physiology , Cerebral Palsy/complications , Fractures, Bone/epidemiology , Fractures, Bone/etiology , Cerebral Palsy/epidemiology , Child , Cross-Sectional Studies , Disabled Persons , Female , Fractures, Bone/diagnosis , Humans , Male , Motor Disorders/epidemiology , Motor Disorders/etiology , Osteoporosis/epidemiologyABSTRACT
Exclusive enteral nutrition (EEN) has been shown to be more effective than corticosteroids in achieving mucosal healing in children with Crohn´s disease (CD) without the adverse effects of these drugs. The aims of this study were to determine the efficacy of EEN in terms of inducing clinical remission in children newly diagnosed with CD, to describe the predictive factors of response to EEN and the need for treatment with biological agents during the first 12 months of the disease. We conducted an observational retrospective multicentre study that included paediatric patients newly diagnosed with CD between 2014-2016 who underwent EEN. Two hundred and twenty-two patients (140 males) from 35 paediatric centres were included, with a mean age at diagnosis of 11.6 ± 2.5 years. The median EEN duration was 8 weeks (IQR 6.6-8.5), and 184 of the patients (83%) achieved clinical remission (weighted paediatric Crohn's Disease activity index [wPCDAI] < 12.5). Faecal calprotectin (FC) levels (µg/g) decreased significantly after EEN (830 [IQR 500-1800] to 256 [IQR 120-585] p < 0.0001). Patients with wPCDAI ≤ 57.5, FC < 500 µg/g, CRP >15 mg/L and ileal involvement tended to respond better to EEN. EEN administered for 6-8 weeks is effective for inducing clinical remission. Due to the high response rate in our series, EEN should be used as the first-line therapy in luminal paediatric Crohn's disease regardless of the location of disease and disease activity.
