RESUMEN
Infants born with esophageal atresia and tracheoesophageal fistula, a complex congenital malformation occurring in 1/2500-4000 live births, may suffer threats to their cardiac, respiratory, and digestive health in addition to anomalies that may exist in the genitourinary and musculoskeletal systems. Optimal care for these patients throughout their lives is best achieved through a coordinated, multidisciplinary approach that our health care system is not always well-equipped to provide. This review, though not exhaustive, highlights the components of care that pertain to initial surgical reconstruction and subsequent diagnosis and management of the complications that are most frequently encountered. Authors from among the many specialties involved in the care of these patients summarize the current best practice with attention to the most recent advances. Assessment and improvement of quality of life and transition to adult specialists as children grow to adulthood is also reviewed.
Asunto(s)
Atresia Esofágica , Enfermedades del Recién Nacido , Fístula Traqueoesofágica , Lactante , Recién Nacido , Niño , Humanos , Fístula Traqueoesofágica/diagnóstico , Fístula Traqueoesofágica/genética , Fístula Traqueoesofágica/cirugía , Atresia Esofágica/complicaciones , Atresia Esofágica/diagnóstico , Atresia Esofágica/genética , Calidad de Vida , Estudios RetrospectivosRESUMEN
Children who undergo ileal pouch anal anastomosis (IPAA) surgery for refractory ulcerative colitis (UC) may ultimately develop a Crohn's disease (CD) phenotype. This de novo CD is open to broad interpretation and misattribution, and its manifestation in children is poorly understood. The surgically altered environment of the ileal pouch is at risk of a spectrum of ileal pouch disorders, which have limited description in children. In this issue of Inflammatory Bowel Diseases, a multicenter, retrospective study of children with UC who underwent IPAA and developed de novo CD highlights the challenges and opportunities of ileal pouch characterization in children.
In this issue of Inflammatory Bowel Diseases, a multicenter, retrospective study provides insight into the poorly understood circumstances in which children who have undergone ileal pouch anal anastomosis surgery for refractory ulcerative colitis develop a Crohn's disease phenotype.
RESUMEN
Classically considered a disease of early childhood characterised by malabsorption and failure to thrive, coeliac disease is now recognised to arise in genetically susceptible individuals at any age. Although permissive HLA genotypes are the strongest predictor of coeliac disease, they are not sufficient. Several prospective cohort studies enrolling genetically at-risk infants have investigated the role of potential triggers of coeliac disease autoimmunity, such as timing of gluten introduction, viral infections and dietary patterns. Much less is known about triggers of coeliac disease in adulthood. Better understanding of factors leading to coeliac disease may be helpful in the management of those with potential coeliac disease (elevated serum celiac antibodies without villous atrophy in the small intestine), many of whom initiate a gluten-free diet without demonstration of villous atrophy. There are a range of clinical presentations of celiac disease in childhood and patterns of coeliac serology, including fluctuation and spontaneous reversion on a gluten-containing diet, vary. There is a current debate over best strategies to manage adults and children with potential coeliac disease to avoid over-treatment and under-treatment. Childhood and adolescence carry unique issues pertaining to the diagnosis and management of coeliac disease, and include nutrition and growth, rescreening, repeat biopsy, dietary adherence concerns and transition to adult care. In conclusion, while coeliac disease has similar pathogenesis and general clinical manifestations in paediatric and adult populations, diagnostic and management approaches need to adapt to the developmental stages.