RESUMEN
BACKGROUND: Post-transplant food allergy (LTFA) is increasingly observed after paediatric liver transplantation (LT). Although the immunopathology of LTFA remains unclear, immunoglobulin (Ig) E seems to be implicated. OBJECTIVE: To study humoral and cellular immunity in paediatric LT patients in search for factors associated with LTFA, and compare with healthy controls (HC) and non-transplant food-allergic children (FA). METHODS: We studied serum Ig levels in 29 LTFA, 43 non-food-allergic LT patients (LTnoFA), 21 FA patients and 36 HC. Serum-specific IgA and IgE against common food allergens in LTFA, IgA1 , IgA2 and joining-chain-containing polymeric IgA (pIgA) were measured. Peripheral blood mononuclear cells were analysed by flow cytometry for B and T cell populations of interest. RESULTS: Serum IgA and specific IgA were higher in LTFA compared to LTnoFA. LTFA patients had the highest proportion of circulating T follicular helper cells (cTfh). The percentage of cTfh correlated positively with serum IgA. Unique in LTFA was also the significant increase in serum markers of mucosal IgA and the decrease in the Th17 subset of CXCR5(-) CD4(+) cells compared to HC. Both LT patients exhibited a rise in IgA(+) memory B cells and plasmablasts compared to HC and FA. CONCLUSIONS: LT has an impact on humoral immunity, remarkably in those patients developing FA. The increase in serum markers of mucosal IgA, food allergen-specific IgA and cTfh cells observed in LTFA, point towards a disturbance in intestinal immune homoeostasis in this patient group.
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Hipersensibilidad a los Alimentos/sangre , Hipersensibilidad a los Alimentos/inmunología , Inmunoglobulina A/inmunología , Trasplante de Hígado , Linfocitos T Colaboradores-Inductores/inmunología , Adolescente , Factores de Edad , Subgrupos de Linfocitos B/inmunología , Subgrupos de Linfocitos B/metabolismo , Biomarcadores , Recuento de Linfocito CD4 , Niño , Preescolar , Femenino , Hipersensibilidad a los Alimentos/diagnóstico , Humanos , Inmunoglobulina A/sangre , Inmunoglobulina A Secretora/inmunología , Inmunoglobulina E/sangre , Inmunoglobulina E/inmunología , Memoria Inmunológica , Inmunofenotipificación , Lactante , Trasplante de Hígado/efectos adversos , Masculino , Linfocitos T Colaboradores-Inductores/metabolismoRESUMEN
PURPOSE: We studied the use of colon enemas in achieving fecal pseudocontinence in patients with spina bifida to define the variables associated with success. MATERIALS AND METHODS: Questionnaires were individually filled out by all patients with spina bifida using colon enemas at our Spina Bifida Reference Center between October 2009 and June 2010. Patient age, type of enema, volume required, evacuation time, followup, continence and independence were recorded. Fecal pseudocontinence was defined as no involuntary stool loss during the last 6 months. Social continence was defined as involuntary stool loss less than once monthly. Children are routinely seen at the reference center, while adults are seen on request. RESULTS: A total of 25 children and 15 adults with spina bifida were studied. Median volume required was 1 liter (range 0.5 to 2) in children and 1.5 liters (0.75 to 3) in adults. Median evacuation time was 30 minutes (range 15 to 60) in children and 60 minutes (30 to 120) in adults. Fecal continence was achieved in 76% of children (19 of 25) and 60% of adults (9 of 15), and social continence in 88% of children (23 of 25) and 67% of adults (10 of 15). A significant relation was found between medical followup since childhood and fecal pseudocontinence. No enema determinants predicted pseudocontinence. CONCLUSIONS: Colon enemas are a valuable method in achieving continence. At our center medical followup from childhood to adulthood is associated with successful acquisition of fecal pseudocontinence.
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Enema , Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Disrafia Espinal/complicaciones , Adolescente , Adulto , Niño , Preescolar , Colon , Humanos , Persona de Mediana Edad , Adulto JovenRESUMEN
BACKGROUND AND STUDY AIMS: Transanal irrigation (TAI) is used in children to treat constipation and incontinence. Belgium has 2 systems available: Colotip® (cheaper, however not designed for TAI) or Peristeen®. PATIENTS AND METHODS: This patient-control switch study is the first to compare 2 TAI systems. Children regularly using Colotip® for TAI were asked to participate, after consent, a visual analogue scale (VAS) rating the system and a 2-week diary (fecal continence, self-reliance, time spent on the toilet, pain, Bristol stool scale, irrigation volume and frequency of enema) were completed. Non-parametric statistics were used. RESULTS: Out of 26 children using Colotip®, 18 (69%) children participated and 5 refused (fear n=1, satisfaction Colotip® system n=7). Of these 18 children (interquartile range: 3-18 years, median 12.5 years, 9 girls) 5 patients stopped Peristeen® (pain n=1, fear n=1 and balloon loss n=3) and 2 were lost from follow up. Dropouts and included patients showed no statistical difference. In the 11 remaining patients, pseudo-continence (p 0.015), independence (p 0.01) and VAS score (p 0.007) were significantly better with Peristeen®, no difference was found in time spent on the toilet (p 0.288) and presence of pain (p 0.785). CONCLUSIONS: In children Peristeen® offered significantly higher pseudo-continence and independency. 30% refused participation because of satisfaction with the Colotip® and 30% spina bifida patients reported rectal balloon loss due to sphincter hypotony. To diminish Peristeen® failure, a test-catheter could be of value. Considering Colotip® satisfaction, both systems should be available. Patient selection for Peristeen® needs further research.
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Incontinencia Fecal , Bélgica , Niño , Estreñimiento , Enema , Femenino , Humanos , Estudios ProspectivosRESUMEN
Cobalamin or vitamin B12 (vitB12) is involved in DNA synthesis, haematopoiesis and myelinisation. Consequently, vitB12 deficiency causes various symptoms, such as megaloblastic anaemia, neurologic signs or pancytopenia. Despite possible severe symptoms, vitB12 deficiency can present asymptomatically. We report six paediatric patients with different aetiologies of vitB12 deficiency ranging from a subtle to a more overt presentation. VitB12 deficiency is a diagnostic challenge due to the lack of consensus on normal values of vitB12 and its co-markers (folate, holotranscobalamin, methylmalonic acid, homocysteine) and the lack in specificity and sensitivity of the serum vitB12 analysis. All cases were treated with parenteral vitB12. Last decades, evidence supporting high dose oral treatment being as effective as the intramuscular (IM) therapy, also in children, is growing.
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Deficiencia de Vitamina B 12 , Biomarcadores , Niño , Pruebas Diagnósticas de Rutina , Ácido Fólico , Humanos , Vitamina B 12 , Deficiencia de Vitamina B 12/diagnóstico , Deficiencia de Vitamina B 12/tratamiento farmacológicoRESUMEN
Scoliosis is a common complication in children with cerebral palsy (CP). In these patients, surgical correction carries a high risk of complications. CP is also associated with gastrointestinal dysmotility such as delayed gastric emptying and gastro-oesophageal reflux. We describe 5 patients with CP in whom symptoms of gastric dysmotility clearly exacerbated after orthopaedic scoliosis surgery. They all showed persisting vomiting, nausea, bloating, weight loss, and anorexia necessitating total parental nutrition and/or jejunal feeding. This intensified nutritional support resulted in weight gain. Symptoms, however, persisted in half of the patients. The aetiology of these gastro-intestinal motility problems following scoliosis surgery remains unclear. Mechanical obstruction needs to be ruled out. Delayed gastric emptying may be due to postprandial antral hypomotility as a consequence of sympathic stimulation. Malnutrition could further aggravate gastrointestinal dysmotility. This complication should be taken into account when surgery for spinal deformities in CP patients is planned, especially in patients with pre-existing gastrointestinal motility problems.
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Parálisis Cerebral/complicaciones , Reflujo Gastroesofágico/epidemiología , Motilidad Gastrointestinal , Complicaciones Posoperatorias/epidemiología , Escoliosis/cirugía , Adolescente , Anorexia/epidemiología , Femenino , Vaciamiento Gástrico , Humanos , Masculino , Náusea/epidemiología , Procedimientos Ortopédicos/métodos , Escoliosis/etiología , Resultado del Tratamiento , Vómitos/epidemiología , Pérdida de PesoRESUMEN
Discontinuation of treatment in children with inflammatory bowel disease (IBD) in long-term remission remains debatable. The risk of relapse is one of the main concerns in the consideration of reduction or cessation of treatment. In 2017 all paediatric IBD patients treated with originator infliximab at the Department of Paediatric Gastroenterology, Ghent University Hospital, were switched to biosimilar Remsima®. Faecal calprotectin, infliximab through levels and antibodies, white cell count, haemoglobin and C-reactive protein were measured before and after switching to biosimilar. In total 21 IBD patients (3 Ulcerative Colitis - 19 CD) between 7 and 15 years old were switched. Three (14%) patients with CD in clinical, biochemical and histological remission had an unmeasurable through level and antibodies for infliximab, after 22 to 82 months of use. Switching to another treatment or cessation was discussed with patients and parents, all 3 patients decided to stop treatment. All 3 are still in clinical remission 21 to 24 months after treatment stop. Six-monthly follow-up is foreseen.
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Biosimilares Farmacéuticos , Enfermedad de Crohn , Enfermedades Inflamatorias del Intestino , Adolescente , Biosimilares Farmacéuticos/uso terapéutico , Niño , Enfermedad de Crohn/tratamiento farmacológico , Sustitución de Medicamentos , Fármacos Gastrointestinales/uso terapéutico , Humanos , Enfermedades Inflamatorias del Intestino/tratamiento farmacológico , Infliximab/uso terapéutico , Estudios Prospectivos , Resultado del Tratamiento , Privación de TratamientoRESUMEN
Most episodes of vomiting, reduced intake and diarrhoea in children can be evaluated and treated without additional tests. However, when the degree of clinical dehydration is not in line with the patient's medical history, other diagnoses should be suspected. In the presence of a hyponatraemic hypochloraemic metabolic alkalosis, cystic fibrosis (CF) should be included in the differential diagnosis, especially if there is failure to thrive even in the absence of respiratory symptoms. Furthermore, young patients diagnosed with CF have a higher risk for an acute electrolyte decompensation caused by increased salt and fluid losses. We present 4 paediatric cases to raise the awareness of electrolyte disturbances in CF patients.
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Alcalosis , Fibrosis Quística , Deshidratación , Hiponatremia , Niño , Fibrosis Quística/complicaciones , Deshidratación/complicaciones , Insuficiencia de Crecimiento , Humanos , Hiponatremia/etiología , VómitosRESUMEN
BACKGROUND/AIMS: In childhood, clinical presentation of intes- tinal polyps is variable. Painless rectal red blood loss is the most common presenting sign. Most polyps are sporadic, isolated and benign. However, it is important to correctly identify exceptions. Rare inherited polyposis syndromes need to be recognized because of their increased risk of intestinal and extra-intestinal malignancies. Furthermore, a correct diagnosis and treatment of rare gastro-intestinal malignancies is crucial. METHODS: Between 2016 and 2018 we encountered 4 different types of intestinal polyps. A database search was performed and patient files were checked for clinical manifestations and histo- pathology. Literature was searched to recapitulate red flags for these syndromes, probability of underlying genetic disorders and diagnostic criteria. RESULTS: Between 2016 and 2018, 28 patients presented at the Ghent University Hospital with 30 juvenile polyps. Furthermore, we diagnosed juvenile polyposis syndrome, Li Fraumeni syndrome and familial adenomatous polyposis (FAP) in 1 patient each, whilst 2 FAP patients were in follow-up. Each of these diagnoses has a different lifetime risk of (extra)-intestinal malignancy and requires a different approach and follow-up. Histopathology and genetic testing play an important role in identifying these syndromes in pediatric patients. CONCLUSION: Although most intestinal polyps in childhood are benign juvenile polyps that require no follow-up, rare inherited syndromes should be considered and correctly diagnosed since adequate follow-up is necessary to reduce morbidity and mortality from both gastrointestinal and extraintestinal complications and malignancies.
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Poliposis Adenomatosa del Colon , Poliposis Intestinal , Pólipos Intestinales , Poliposis Adenomatosa del Colon/diagnóstico , Poliposis Adenomatosa del Colon/genética , Adolescente , Niño , Pruebas Genéticas , Humanos , Poliposis Intestinal/diagnóstico , Poliposis Intestinal/genética , Pólipos Intestinales/diagnóstico , Pólipos Intestinales/genéticaRESUMEN
AIM: This study evaluates hepatitis B virus (HBV) vaccination response in children with celiac disease (CD). Response in initial non-responders after a single booster vaccination as well as factors influencing HBV vaccination response were evaluated. METHODOLOGY: Anti-hepatitis B surface antibodies (a-HBsAB) were checked in all children with CD and a documented complete HBV vaccination. An a-HBsAB <10 U/L was considered as non-response. A single intramuscular HBV-vaccine booster was advised to all non-responders. Response was checked at the next appointment. RESULTS: 133 children with CD were included, median age of 7.3 years (range 1.7-17.3) and 46 (35%) were male. The age at CD diagnosis was 6.0 years (range 1.1-15.7). HBV non-response was documented in 55% (n=73/133). No other factors were influencing the response. A booster was documented in 34/73 (47 %) initial non-responders (3 refused (4%), 36 (49%) had no follow up). Response after booster vaccination resulted in immunity in 22/34 (65%) and persisting non-response in 12/34 (35%). A single booster is able to reduce non-response from 55% (73/133) to 23% (22/94). CONCLUSION: A significantly lower immune response following HBV vaccination in children with CD was confirmed. A single intramuscular booster vaccination is able to induce a serologic response in two thirds of the initial non-responders. Control of HBV vaccination response has to become part of the follow-up in CD patients.
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Enfermedad Celíaca , Vacunas contra Hepatitis B/administración & dosificación , Hepatitis B/prevención & control , Vacunación/estadística & datos numéricos , Adolescente , Enfermedad Celíaca/sangre , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/inmunología , Niño , Preescolar , Hepatitis B/complicaciones , Hepatitis B/inmunología , Anticuerpos contra la Hepatitis B/análisis , Anticuerpos contra la Hepatitis B/sangre , Antígenos de Superficie de la Hepatitis B/metabolismo , Antígenos de Superficie de la Hepatitis B/uso terapéutico , Vacunas contra Hepatitis B/metabolismo , Vacunas contra Hepatitis B/uso terapéutico , Virus de la Hepatitis B , Humanos , Inmunidad Activa/efectos de los fármacos , Inmunización Secundaria , Huésped Inmunocomprometido/efectos de los fármacos , Lactante , Masculino , Estudios ProspectivosRESUMEN
AIM: To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. METHODS: A retrospective study of all CF patients treated with Zn supplements (January 2002 to December 2004). Data from patient files for the year before and the first year of supplementation were compared. The controls were CF patients with normal serum Zn and without Zn supplementation. RESULTS: 21 patients (7 females), median age 8.9 (interquartile range 13.1) years, received 5 mg/kg Zn sulfate/day (maximum 150 mg). The number of infections decreased from 3 (1.25) to 2 (2.0) (tied p < 0.02) and the forced expiratory volume in 1 s (FEV(1)) increased from 72.0 (38.4) to 76.5 (52)% (p < 0.02). Energy intake improved (92.3 (14.5) to 117.0 (28.5)%; tied p < 0.02), as did weight for height (W/H; 90 (9.4) to 94 (8.5)%; tied p = 0.043). In the CF control patients the number of infections (2.0 (2.0)), energy intake (116 (43.3)%) and nutritional status remained stable (W/H 99 (17.2)%), but pulmonary function decreased significantly (DeltaFEV(1) loss of 2.0 (8.0)%). There was a significantly different evolution for the change in forced vital capacity (p < 0.004) and DeltaFEV(1) (p < 0.001) between supplemented and control patients. CONCLUSIONS: Analysis of the clinical data on Zn supplementation in CF showed beneficial effects in Zn-deficient CF patients. These results must be confirmed in a prospective double-blind randomized control trial.
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Fibrosis Quística/complicaciones , Suplementos Dietéticos , Esteatorrea/etiología , Zinc/deficiencia , Zinc/uso terapéutico , Adolescente , Apetito/efectos de los fármacos , Niño , Preescolar , Susceptibilidad a Enfermedades , Ingestión de Energía/efectos de los fármacos , Insuficiencia Pancreática Exocrina/etiología , Femenino , Volumen Espiratorio Forzado , Trastornos del Crecimiento/etiología , Humanos , Infecciones/epidemiología , Masculino , Estudios Retrospectivos , Albúmina Sérica/análisis , Esteatorrea/metabolismo , Delgadez/etiología , Capacidad Vital , Vitaminas/sangre , Zinc/sangreRESUMEN
BACKGROUND AND STUDY AIMS: Constipation and fecal incontinence are common problems in neurologically impaired children. This paper aims to give an overview on bowel problems in cerebral palsy children and to suggest a stepwise treatment approach. A pubmed search was performed looking at studies during the past 20 years investigating bowel problems in neurologically disabled children. RESULTS: The search revealed 15 articles. Prevalence and presentation was the subject of 8 papers, confirming the importance of the problem in these children. The other papers studied the results of different treatment modalities. No significant differences between treatment modalities could be demonstrated due to small studied cohorts. Therefore, no specific treatment strategy is currently available. An experienced based stepwise approach is proposed starting with normalization of fiber intake. The evaluation of the colon transit time could help in deciding whether desimpaction and eventually laxatives including both osmotic (lactulose, macrogol) as well as stimulant laxatives might be indicated. Or, in case of fast transit loperamide or psyllium can be tried. Surgery should be a last resort option. CONCLUSION: Studies investigating constipation and continence in neurologically impaired children are scarce, making it difficult to choose for the optimal treatment. A stepwise treatment approach is proposed, measuring the colon transit time to guide treatment choices.
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Parálisis Cerebral/epidemiología , Estreñimiento/epidemiología , Incontinencia Fecal/epidemiología , Antidiarreicos/uso terapéutico , Catárticos/uso terapéutico , Niño , Estreñimiento/tratamiento farmacológico , Estreñimiento/fisiopatología , Incontinencia Fecal/tratamiento farmacológico , Incontinencia Fecal/fisiopatología , Fármacos Gastrointestinales/uso terapéutico , Tránsito Gastrointestinal , Humanos , Lactulosa/uso terapéutico , Laxativos/uso terapéutico , Loperamida/uso terapéutico , Polietilenglicoles/uso terapéutico , Prevalencia , Psyllium/uso terapéuticoRESUMEN
PURPOSE: Fecal incontinence is a major problem in patients with myelomeningocele. We evaluate the results of a stratified approach aimed at obtaining fecal pseudo-continence in patients with myelomeningocele. MATERIALS AND METHODS: We conducted a cross-sectional descriptive study of last file data in 80 patients 5 to 18 years old with myelomeningocele followed at our center. Beginning at birth patients with myelomeningocele were seen at least annually by the pediatric gastroenterologist, a member of the multidisciplinary "spina team." Constipation was treated with diet and osmotic laxatives. Starting at age 5 years, treatment was targeted at achieving pseudo-continence. A toilet training scheme was started, associated with induced defecation by digital stimulation. Retrograde tap water enemas were used in patients with unsatisfactory results. If retrograde enemas were unsuccessful, an antegrade continence enema procedure was proposed. For children unable to sit on a toilet regular manual evacuation of stools was advised. RESULTS: Eight of the 80 patients were fecal continent. Pseudo-continence was achieved in 50 of the 72 incontinent patients (69%), including 5 of 5 following only a strict toilet scheme, 21 of 24 (87.5%) performing retrograde enemas, 16 of 20 (80%) performing orthograde enemas through an antegrade continence device and 8 of 10 performing regular manual evacuation of stools. In 4 of the 20 patients (20%) performing orthograde enemas complications led to closure of the antegrade continence device. Treatment failed and was stopped in 17 patients. Success of treatment was not related to level of spinal lesion or degree of mobility. CONCLUSIONS: Fecal pseudo-continence was achieved in 58 of 80 patients (72.5%) with myelomeningocele.
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Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Disrafia Espinal/complicaciones , Adolescente , Canal Anal , Niño , Preescolar , Estudios de Cohortes , Terapia Combinada , Estudios Transversales , Defecación/fisiología , Tacto Rectal/métodos , Enema/métodos , Incontinencia Fecal/fisiopatología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estimulación Física/métodos , Factores de Riesgo , Disrafia Espinal/diagnósticoRESUMEN
AIM: Assess the risk of zinc (Zn) deficiency in the older cystic fibrosis (CF) population. METHOD: Cross-sectional investigation of all CF patients above the age of 4 followed at the Ghent University center between 2002 and 2003. Data on age, weight, height z-score, pancreatic and pulmonary functions, chronic Pseudomonas infection, and CF transmembrane conductance regulator (CFTR) mutations were collected. Serum Zn, vitamins (vit) A and E, retinol-binding protein (RBP), albumin, sedimentation rate, total IgG, and cholesterol were determined. Serum Zn was compared with a local healthy control group (Van Biervliet et al., Biol Trace Elem Res 94:33-40, 2003) and with literature data (Hotz C, et al. Am J Clin Nutr 78:756-764, 2003). RESULTS: 101 patients (median age 16 years) were included. There was no difference in serum Zn concentration between CF patients and controls. In CF patients no difference in serum Zn concentration between pancreatic-sufficient or pancreatic-insufficient patients was seen. Serum Zn was not associated to nutritional status or height z-score. A significant association serum Zn to serum albumin (p < 0.0005) and to vit A (p < 0.01) was seen. No associations of serum Zn to serum vit E, RBP, cholesterol, or CFTR were present, but there is a significant association serum Zn to forced vital capacity (p < 0.01). Serum Zn was not associated to inflammatory parameters or chronic Pseudomonas infection. CONCLUSION: Comparison of CF patients with local controls revealed no significant differences. However, because persisting steatorrhea increases Zn loss (Easley et al., J Pediatr Gastroenterol Nutr 26:136-139, 1998) and 12.6% of our population has a serum Zn below the p value of 2.5 of the NHANES II study (Hotz C, et al. Am J Clin Nutr 78:756-764, 2003), there could remain an increased risk of Zn deficiency in some CF patients. Furthermore, the association with pulmonary function needs more investigation.
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Fibrosis Quística/sangre , Zinc/sangre , Zinc/deficiencia , Adolescente , Niño , Preescolar , Estudios Transversales , Fibrosis Quística/fisiopatología , Enfermedades Carenciales/diagnóstico , Femenino , Humanos , Masculino , Infecciones por Pseudomonas/fisiopatología , Pruebas de Función RespiratoriaRESUMEN
Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disease of unknown etiology, most commonly affecting the metaphysis of long bones, especially the tibia, femur and clavicle. The clinical spectrum varies from self-limited uni-or multi-focal lesions to chronic recurrent courses. Diagnosis is based on clinical, radiologic and pathological findings, is probably underdiagnosed due to poor recognition of the disease. A dysregulated innate immunity causes immune cell infiltration of the bones with subsequent osteoclast activation leading to sterile bone lesions. The molecular pathophyiology is still incompletely understood but association with other auto-inflammatory diseases such as inflammatory bowel disease (IBD), psoriasis, Wegener's disease, arthritis and synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is interesting. CRMO can precede the symptoms of the associated disease by several years. The bone remodeling caused by CRMO can cause permanent disability. We report the case of a 10-year-old boy with CRMO in association with Crohn's disease.
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Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/patología , Osteomielitis/diagnóstico , Osteomielitis/etiología , Niño , Humanos , MasculinoRESUMEN
AIM: Monocentric retrospective paediatric study describing indications for gastrostomy and major complications, compared to literature data as part of a quality check. METHODS: Records of all gastrostomy patients consulting at the UZ Ghent paediatric gastro-enterology department between January 2007-December 2009 were reviewed in December 2010 regarding indication, age and weight at tube insertion, insertion method, major complications and current gastrostomy tube type. RESULTS: 178 patients were included of which 165 (93%) were placed using the endoscopic pull technique, the others were placed surgically (n = 13). Neurodevelopmental disability with oral motor dysfunction was the major indication (113, 63%). Other indications were failure to thrive due to concomitant disease (65, 37%). Median age at tube insertion was 3yr (interquartile range (IQR) 0.6-9) with median tube time of 3.9 yr (IQR 1.9-7.2). Immediate complications were 1 peritonitis and 1 post-insertion fever episode. Late complications (10, 5.6%) were 1 gastrocolic fistula, 1 dislocation and 8 buried bumpers after 4 yr (range 35-10.4) of tube insertion. The incidence of buried bumper increased significantly with increasing PEG tube time (P < 0.01). Gastro-oesophageal reflux disease (GORD) led to Nissen fundoplication in 45 (25.3%) patients. The proportion of patients receiving a fundoplication remained about 20% over time but the time lapse between the 2 procedures decreased significantly. CONCLUSION: The development of buried bumper is associated to prolonged PEG tube use. In case of important GORD laparoscopic Nissen procedure and PEG placement can be performed simultaneously without increasing complication rate.
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Enfermedades del Sistema Digestivo/cirugía , Nutrición Enteral , Gastrostomía , Adolescente , Factores de Edad , Niño , Preescolar , Enfermedades del Sistema Digestivo/etiología , Humanos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Feeding difficulties, mainly determined by oral motor problems, are common in patients with severe neurodevelopmental disabilities (NDD). These problems have a negative impact on health and developmental outcome as a consequence of insufficient intake. Research data show that insufficient intake, and not enhanced caloric needs, is the major cause of malnutrition. However, no direct relation between intake and nutritional state has been shown, illustrating the wide variety in caloric needs in this population. Individual caloric needs in patients with NDD show a wide variety, but are generally lower than in normal children. Treatment of these patients is complex as data on adequate daily allowances for this population are not available and standard anthropometric cut-off points to define malnutrition need adaptation. In order to prevent and treat malnutrition in patients with neurodevelopmental problems, careful multidisciplinary follow-up is indicated, aimed at early detection of feeding problems, nutritional deficiencies and growth failure. Oral food intake can be enhanced using adapted food texture and special feeding devices, giving positional support combined with specialized dietary advice on nutrient- and caloric-dense food. When oral feeding is unsafe or inefficient, partial or total enteral nutrition is started through a gastrostomy, with or without concomitant fundoplication. Evidence based criteria guiding this decision are lacking.
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Discapacidades del Desarrollo/terapia , Estado Nutricional , Apoyo Nutricional/métodos , Bélgica , Niño , HumanosRESUMEN
INTRODUCTION: Patients with open spinal dysraphism (OSD) frequently present constipation and incontinence requiring treatment. AIM: Evaluation of colon transit time (CTT) in patients with OSD, in relation to neural lesion, mobility, bowel habits, and continence status. METHODS: OSD patients aged between 6 and 20 years, who did not use antegrade enemas, were invited to participate in the study. Data from the medical file and information retrieved by questionnaires for constipation and incontinence were collected. The control group consisted of 13 healthy age-matched children. CTT was measured using the 6-day pellet method with an abdominal X-ray on day 7. Laxatives were continued and retrograde colon enemas were stopped 48h prior the X-ray. RESULTS: Thirty of the 33 patients who met the inclusion criteria agreed to participate. Twelve (40%) patients were constipated (Rome III criteria) despite treatment. Fifteen (50%) were continent, with or without treatment. Total CTT was significantly longer in OSD patients (median CTT: 86.4h vs. 43.2h controls). Constipated OSD patients had a significantly prolonged CTT compared to non-constipated patients (CTT: 125.4h vs. 51.6h). Spontaneous continent OSD patients had a normal CTT (CTT: 33.6h). An abnormal CTT predicted the necessity of treatment to achieve continence (P<0.006). CONCLUSION: CTT in OSD patients is significantly prolonged, indicating a neurogenic involvement of the bowel and a slow transit constipation. An abnormal CTT predicts the necessity of therapy to achieve fecal continence.
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Colon/fisiopatología , Tránsito Gastrointestinal/fisiología , Espina Bífida Quística/fisiopatología , Adolescente , Niño , Colon Ascendente/fisiopatología , Colon Descendente/fisiopatología , Colon Sigmoide/fisiopatología , Estreñimiento/fisiopatología , Defecación/fisiología , Enema , Impactación Fecal/fisiopatología , Incontinencia Fecal/fisiopatología , Femenino , Humanos , Laxativos/uso terapéutico , Masculino , Estudios Prospectivos , Factores de Tiempo , Adulto JovenRESUMEN
Anal canal duplication (ACD) is the least frequent digestive duplication. Symptoms are often absent but tend to increase with age. Recognition is, however, important as almost half of the patients with ACD have concomitant malformations. We present the clinical history of an eleven-year-old girl with ACD followed by a review of symptoms, diagnosis, treatment, and prognosis based on all the reported cases in English literature.
RESUMEN
The paediatric population aged 1-3 years represents about 80% of patients presenting with foreign body ingestion. Only 10 to 20% of ingested foreign bodies will fail to pass through the entire gastrointestinal tract. The decision to remove them is based on location, size and nature of the foreign body. In particular, ingestion of multiple magnets or magnets and other metallic objects require a more aggressive management.
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Cuerpos Extraños/cirugía , Intestinos , Imanes , Juego e Implementos de Juego , Niño , Ingestión de Alimentos , Humanos , Perforación Intestinal/etiología , Perforación Intestinal/cirugía , Intestinos/cirugía , MasculinoRESUMEN
Myotonic dystrophy or Steinert disease is an autosomal dominant multisystemic disorder with variable penetrance. The genetic defect is an amplified trinucleotide repeat in the 3-prime untranslated region of a proteinkinase gene on chromosome 19. Severity of symptoms increases with the number of repeats. Patients with myotonic dystrophy often present with gastrointestinal motility problems, such as intermittent diarrhoea, constipation, and also faecal incontinence. The underlying physiopathological mechanism of faecal incontinence differs from classic soiling due to faecal retention. We present a girl with congenital myotonic dystrophy and faecal incontinence due to anal sphincter atrophy; and give an overview of present knowledge on the pathophysiology of gastrointestinal problems associated with myotonic dystrophy.