RESUMEN
BACKGROUND AND AIMS: Incidence of paediatric inflammatory bowel disease [IBD] in Canada is among the highest worldwide, and age of onset may be decreasing. In a multicentre nationwide inception cohort study, we examined variation in phenotype of IBD throughout the paediatric age spectrum. METHODS: Children aged ≥2 years [y] and <17y [A1 age at diagnosis], with new onset IBD, were systematically evaluated at sites of the Canadian Children IBD Network. Prospectively recorded phenotypic data were compared between age groups. RESULTS: Among 1092 children (70% Caucasian; 64% Crohn's disease [CD], 36% ulcerative colitis/inflammatory bowel disease unclassified [UC/IBD-U]; median age 13 y, interquartile range [IQR] 11-15 y), 210 [19%] were diagnosed before the age of age 10 y [Paris A1a] and 43 [4%] before age 6 y (very-early-onset [VEO-IBD]). CD was less common in younger children [42%, 56%, 66%, respectively, of VEO-IBD, A1a; A1b]. Colon-only IBD [UC/IBDU or CD-colon] was present in 81% of VEO-IBD and 65% of A1a; ileal disease increased progressively, reaching plateau at age 10 y. CD location was ileocolonic [L3] in 53% overall. Ileitis [L1] increased with age [6% of VEO-IBD; 13% of A1a; 21% of A1b], as did stricturing/penetrating CD [4% of A1a; 11% of A1b]. At all ages UC was extensive [E3/E4] in >85%, and disease activity moderate to severe according to Physician's Global Assessment [PGA] and weighted Paediatric Crohn's Disease Activity Index/Paediatric Ulcerative Colitis Activity Index [wPCDAI/PUCAI] in >70%. Heights were modestly reduced in CD [mean height z score -0.30 ± 1.23], but normal in UC/IBD-U. CONCLUSIONS: Paris classification of age at diagnosis is supported by age-related increases in ileal disease until age 10 years. Other phenotypic features, including severity, are similar across all ages. Linear growth is less impaired in CD than in historical cohorts, reflecting earlier diagnosis.
Asunto(s)
Colitis Ulcerosa , Enfermedad de Crohn , Edad de Inicio , Variación Biológica Poblacional , Canadá/epidemiología , Niño , Estudios de Cohortes , Colitis Ulcerosa/diagnóstico , Colitis Ulcerosa/epidemiología , Colitis Ulcerosa/fisiopatología , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/epidemiología , Enfermedad de Crohn/fisiopatología , Progresión de la Enfermedad , Femenino , Humanos , Incidencia , Masculino , Estudios Prospectivos , Índice de Severidad de la EnfermedadRESUMEN
Suboptimal vitamin D (vitD) status and reduced lean body mass are highly prevalent in pediatric inflammatory bowel diseases (IBD). The study objective was to determine sarcopenia prevalence and associations with vitD status in newly diagnosed pediatric IBD. Children with Crohn's disease (CD; n = 58) and ulcerative colitis (UC; n = 27) were included. Primary outcomes included body composition (total/regional/percent fat mass (FM), fat-free mass (FFM), skeletal muscle mass (SMM)), and vitD status (serum 25(OH)D). Sarcopenia was defined as SMM-z < -2. Additional variables measured included serum CRP, ESR, anthropometric, Pediatric Crohn's Disease Activity Index (PCDAI), and the Pediatric Ulcerative Colitis Disease Activity index (PUCAI). Sarcopenia and suboptimal 25(OH)D levels (< 50 nmol/l) were found in 23.5% (n = 20) and 52% (n = 44) of children, respectively. Younger children (< 13 years) with CD with suboptimal 25(OH)vitD (< 50 nmol/l) had the greatest frequency of sarcopenia (57.1%) (p = 0.004). Sarcopenia was prevalent in newly diagnosed, young children with CD with vitD deficiency.
Asunto(s)
Enfermedades Inflamatorias del Intestino , Sarcopenia , Deficiencia de Vitamina D , Vitamina D/sangre , Adolescente , Niño , Preescolar , Estudios de Cohortes , Humanos , Enfermedades Inflamatorias del Intestino/complicaciones , Enfermedades Inflamatorias del Intestino/epidemiología , Estado Nutricional , Factores de Riesgo , Sarcopenia/complicaciones , Sarcopenia/epidemiología , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/epidemiologíaRESUMEN
BACKGROUND: Patients with inflammatory bowel disease (IBD) often do not take their medications as prescribed. OBJECTIVE: To examine self-reported adherence rates in IBD patients at the Stollery Children's Hospital (Edmonton, Alberta) and to determine predictors of medication adherence. METHODS: A survey was mailed to 212 pediatric IBD patients of the Stollery Children's Hospital. A chart review was completed for those who returned the survey. RESULTS: A total of 119 patients completed the survey. The nonresponders were significantly older than responders (14.5 years versus 13.2 years; P=0.032). The overall adherence rate was 80%. Nonadherence was associated with older age (14.6 years versus 13.0 years; P=0.04), longer disease duration (5.0 years versus 3.1 years; P=0.004) and reported use of herbal medications (40.0% versus 13.6%; P=0.029). The most common reasons reported for missing medications were forgetfulness, feeling better and too many medications. In addition, patients reported being more likely to take anti-inflammatory medications and less likely to take herbal medicines. CONCLUSION: Identified predictors of nonadherence such as age, disease duration and use of herbal treatments may enable the development of specific strategies to improve adherence in adolescents with IBD.
Asunto(s)
Enfermedades Inflamatorias del Intestino/tratamiento farmacológico , Cumplimiento de la Medicación , Fitoterapia , Adolescente , Factores de Edad , Alberta , Antiinflamatorios/uso terapéutico , Niño , Estudios Transversales , Recolección de Datos , Femenino , Hospitales Pediátricos , Humanos , Masculino , Factores de TiempoRESUMEN
Intestinal failure (IF) is a complex, chronic illness, of increasing importance in the pediatric critical care setting. We can expect an increase in pediatric IF given an increase in the survivors of extreme prematurity and complex congenital heart disease. Overall priorities for management of this condition include surgical and medical strategies to promote intestinal adaptation and to reduce complications, particularly related to malnutrition, liver disease and sepsis. In this review the authors propose that the optimal care for children with IF are multidisciplinary teams abreast of the newest strategies for intestinal rehabilitation. Early listing for intestinal transplantation for children at greatest risk of long-term parenteral nutrition dependency and its life threatening complications is appropriate.
Asunto(s)
Cuidados Críticos , Síndromes de Malabsorción , Niño , Tratamiento de Urgencia/métodos , Humanos , Intestinos/trasplante , Hepatopatías/prevención & control , Síndromes de Malabsorción/complicaciones , Síndromes de Malabsorción/etiología , Síndromes de Malabsorción/mortalidad , Síndromes de Malabsorción/terapia , Desnutrición/prevención & control , Grupo de Atención al Paciente , Factores de Riesgo , Sepsis/prevención & control , Resultado del TratamientoRESUMEN
AIMS: To evaluate the efficacy of bovine lactoferrin (BLf), recombinant human lactoferrin (rHLf) and desferrioxamine against Helicobacter pylori in vitro and in mice and also to determine whether BLf or rHLf alter gastric inflammation. METHODS AND RESULTS: In vitro: Broth dilution susceptibility tests were performed using different concentrations of desferrioxamine, BLf and rHLf. Murine trials: In the prevention trial, C57BL/6 female mice were treated with BLf or rHLF, and then infected with the SS1 strain of H. pylori. In the treatment trial, mice were gavaged with either BLf, rHLf or desferrioxamine. In addition, gastric myeloperoxidase activity (MPO) was measured to assess gastric inflammation. Desferoxamine was found to have a direct bactericidal effect, while BLf and rHLf only partially suppressed H. pylori growth in vitro. However, in both prevention and treatment trials all three forms of treatment failed to reduce H. pylori load in mice. Gastric MPO activity and H. pylori load were noted to be higher with lactoferrin treatments. CONCLUSIONS: Our study does not support the use of BLf or rHLF in the treatment of human H. pylori infection. Interestingly, H. pylori growth and gastric inflammation appear to be enhanced by lactoferrin treatment. SIGNIFICANCE AND IMPACT OF THE STUDY: The mouse model is ideal for testing novel H. pylori eradicating agents.
Asunto(s)
Antibacterianos/farmacología , Deferoxamina/farmacología , Mucosa Gástrica/inmunología , Infecciones por Helicobacter/tratamiento farmacológico , Helicobacter pylori/crecimiento & desarrollo , Lactoferrina/farmacología , Animales , Antibacterianos/efectos adversos , Deferoxamina/efectos adversos , Femenino , Mucosa Gástrica/efectos de los fármacos , Mucosa Gástrica/microbiología , Infecciones por Helicobacter/inmunología , Infecciones por Helicobacter/microbiología , Helicobacter pylori/efectos de los fármacos , Humanos , Inflamación/tratamiento farmacológico , Inflamación/microbiología , Lactoferrina/efectos adversos , Ratones , Resultado del TratamientoRESUMEN
We report on two Aboriginal patients with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome. Both presented with acute hepatic failure with severe hypertransaminasemia and coagulopathy, prompting evaluation for emergent liver transplantation. The diagnosis of HHH syndrome was based on the presence of typical metabolic abnormalities. A protein-restricted diet and L-arginine or L-citrulline supplementation were immediately started, with rapid normalization of liver function test results and other biochemical abnormalities. Molecular analysis of the SLC25A15 gene showed that the two patients were homozygous for the common French Canadian mutation (F188Delta). The diagnosis of HHH syndrome should be considered in patients with unexplained fulminant hepatic failure. There does not appear to be a genotype-phenotype correlation for this presentation, inasmuch as the only other reported patient presenting with this picture had two different point mutations. Early identification and prompt treatment of these patients is crucial to avoid liver transplantation and can be life saving.
Asunto(s)
Dieta con Restricción de Proteínas , Hiperamonemia/complicaciones , Fallo Hepático Agudo/etiología , Errores Innatos del Metabolismo/complicaciones , Mutación Puntual , Sistemas de Transporte de Aminoácidos Básicos , Citrulina/análogos & derivados , Citrulina/sangre , Citrulina/orina , Femenino , Humanos , Hiperamonemia/sangre , Hiperamonemia/genética , Lactante , Trasplante de Hígado , Errores Innatos del Metabolismo/sangre , Errores Innatos del Metabolismo/genética , Proteínas de Transporte de Membrana Mitocondrial , Ornitina/sangre , Ornitina/orina , SíndromeRESUMEN
We report a successful dilation of a completely obstructed distal esophageal stricture in a 4-year-old boy with combined immune deficiency syndrome, at 2 and half years after fundoplication and gastrostomy tube insertion. Barium studies and esophagoscopy had revealed complete obstruction of the lower esophagus. Transgastrostomy gastroscopy demonstrated a pinhole lumen through the fundoplication wrap; a guide wire was passed into the esophagus; and the stricture was dilated with Savary dilators. We presumed that the stricture was secondary to chronic esophagitis. The stricture was identified and successfully dilated using a novel technique of concurrent esophagoscopy and transgastrostomy gastroscopy.
Asunto(s)
Dilatación/métodos , Estenosis Esofágica/terapia , Esofagitis Péptica/complicaciones , Esofagoscopía/métodos , Fundoplicación , Gastroscopía/métodos , Complicaciones Posoperatorias/terapia , Preescolar , Estenosis Esofágica/etiología , Gastrostomía , Humanos , MasculinoRESUMEN
BACKGROUND: Mucosal damage by H. pylori infection is mainly caused by neutrophils producing large quantities of reactive oxygen species (ROS). Metallothionein (MT) an intracellular, low-molecular, cysteine-rich protein, which is inducible by dietary zinc (Zn), has been implicated in sequestering ROS. This study examines the effects of Zn supplementation on Helicobacter colonisation and associated gastritis and the relationship with gastric MT levels. METHODS: C57Bl/6 mice were inoculated with either 10(8) H. pylori or H. felis and were infected for 4 weeks or 6 and 12 weeks, respectively. Mice infected with H. pylori (4 weeks) or H. felis (6 weeks) were treated with either Zn acetate (ZnA; 1 mg/ml), or Zn sulphate (ZnSO4; 5 mg/ml) for 2 weeks with 0.1 ml oro-gastric gavage twice daily. H. pylori load and H. felis colonisation density were determined by culture and microscopy, respectively. MT levels and H. felis-induced gastritis were also determined. RESULTS: Zn treatment showed no significant difference in Helicobacter load and gastric MT, however, ZnSO4 treatment showed a significant (p<0.05) increased in gastric MT in H. felis infected mice. Both Zn-treated groups showed a significant (p<0.05) difference in gastritis score in the antrum of the stomach within the basal and submucosal compartments compared to H. felis-infected controls. CONCLUSIONS: We found that H. felis-induced gastritis can be attenuated by short-term treatment of Zn. This observation suggests that Zn alone may be effective for the suppression of gastric mucosal inflammation induced by Helicobacter.