IMPACT-III and IMPACT-III-P are health-related quality of life (HRQoL) questionnaires for patients with pediatric inflammatory bowel disease (p-IBD) and their parents/caregivers. We aimed to perform ...a transcultural adaptation and validation for the Spanish context. Translation, back-translation, and evaluation of the questionnaires were performed by an expert committee and 12 p-IBD families. We recruited p-IBD patients aged 10–17 and their parents/caregivers. Utility, content, and face validity were considered. Validation was performed with Cronbach’s alpha coefficient and varimax rotation. We confirmed the adequacy of the factor analysis using Kaiser–Meyer–Olkin (KMO) and Bartlett’s sphericity tests. A confirmatory factor analysis was performed using the following goodness indexes: chi-square, Normed Fit Index (NFI), Root Mean Square Error of Approximation index (RMSEA), Standardized Root Mean Square Residual (SRMR), and Comparative Fit Index (CFI). The correlation coefficient between IMPACT-III and IMPACT-III-P was analyzed. We included 370 patients and 356 parents/caregivers (37 hospitals). Both questionnaires had good content and face validity and were considered user-friendly. The KMO measure (0.8998 and 0.9228, respectively) and Bartlett’s sphericity test (
p
-value < 0.001 for both) confirmed the adequacy of the factor analysis. The 4-factor model, complying with Kaiser’s criterion, explained 89.19% and 88.87% of the variance. Cronbach’s alpha (0.9123 and 0.9383) indicated excellent internal consistency. The CFA showed an adequate fit (NFI 0.941 and 0.918, RMSEA 0.048 and 0.053, SRMR 0.037 and 0.044, and CFI 0.879 and 0.913). The correlation coefficient was excellent (0.92).
Conclusion
: The SEGHNP versions of IMPACT-III and IMPACT-III-P are valid and reliable instruments for Spanish p-IBD families.
What is Known:
• IMPACT-III and parent-proxy IMPACT-III (IMPACT-III-P) are useful questionnaires for assessing health-related quality of life (HRQoL) in pediatric inflammatory bowel disease (p-IBD) patients and their parents/caregivers and have been translated and validated in several countries.
• To date, no transcultural adaptation and validation of these questionnaires have been published for Spanish patients with p-IBD and their families.
What is New:
• This is the first transcultural adaptation and validation of IMPACT-III and IMPACT-III-P for Spanish p-IBD families.
• These are valid and reliable instruments for assessing HRQoL in Spanish families of patients with p-IBD.
RESUMEN El síndrome de enterocolitis inducido por proteínas de los alimentos (FPIES, por su sigla en inglés) es una reacción alérgica no mediada por inmunoglobulina E (IgE) con síntomas ...gastrointestinales, como vómitos y diarrea. El diagnóstico se basa en criterios clínicos y en una prueba de provocación para confirmarlo. Es una enfermedad desconocida en las unidades neonatales, debido a la inespecificidad de los síntomas en los recién nacidos. La cifra de metahemoglobina elevada es una opción sencilla de aproximación diagnóstica. Se describe el caso clínico de un recién nacido que ingresa al servicio de urgencias por deshidratación, letargia, vómitos, diarrea y acidosis metabólica grave con elevación de metahemoglobina, con mejora clínica y recuperación total tras el inicio del aporte de fórmula elemental. La sospecha diagnóstica se confirmó tras la prueba de provocación positiva.
Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated allergic reaction with gastrointestinal symptoms, such as vomiting and diarrhea. FPIES diagnosis is based on clinical ...criteria and on a food challenge test. It is an unknown disease in neonatal units due to its nonspecific symptoms in newborn infants. An elevated methemoglobin level is a simple way to approach diagnosis. Here we describe a clinical case of a newborn admitted to the emergency department because of dehydration, lethargy, vomiting, diarrhea, severe metabolic acidosis, and a high methemoglobin level. Clinical improvement and complete recovery was achieved after initiation of elemental formula. The diagnostic suspicion was confirmed after a positive challenge test.
INTRODUCTIONThere are few studies of the intestinal graft function in medium and long term.
OBJECTIVESAssess the functional status of the graft in transplantation patients, once the immediate ...post-transplant has been overcome.
METHODOLOGYPatients who received a liver-intestinal or multivisceral transplantation were prospectively evaluated through cross-sectional data collection during a review. Clinical, analytical and functional variables are analyzed. Unstable patients with intercurrent processes were excluded.
RESULTSTwenty-six patients were analyzed, 65.38% of them male. 21 with multivisceral graft (80.76%) and 5 liver-intestinal (19.23%). 7 multivisceral grafts were retransplantation (26.92%). The average age at transplantation was 1.73 + / 3.22 years (range7 months - 13 years). The average time post-transplant was 3.78 +/- 3.69 years (range 1.5 - 14 years). The indication for transplant was volvulus (19.23%), necrotizing enterocolitis (15.38%), gastroschisis (15.38%), pseudo-obstruction (11.53%), atresia (7.69%) intestinal ischemia (7.69%), epithelial dysplasia (7.69%), MartínezFrías syndrome (3.84%), mitochondrial disease (3.84%) and Hirschsprungʼs disease (3.84%). 96.15% of patients are autonomous oral-enteral. 1 (3.85%) maintains home parenteral nutrition. 15.38% receive enteral nocturnal nutrition. 3 patients (11.53%) maintain ostomy.Fecal alpha 1-antitrypsin was normal in 92.30% of patients. Fecal elastase was normal at 100%. Faecal fat excretion was normal in 84.61% of patients, only 4 (15.38%) with a moderate steatorrhea. Blood parameters were normal in 100% of patients, including serum citrulline levels with a mean of 37.16 μmol / L (range 21-74).
CONCLUSION1. Patients with liver-intestinal and multivisceral transplantation have good graft function in medium and long term.2. Most maintain digestive autonomy.3. Although in some patients steatorrhea is observed, pancreatic function is normal in 100% of patients with a multivisceral transplantation that includes a pancreatic graft.
OBJECTIVEAnalyze the role of spleen in pediatric intestinal/multivisceral transplantation and its variants.
MATERIAL AND METHODSWe analyzed pediatric patients transplanted in our unit from Octoberʼ99 ...to Mayʼ15. Comorbidities (cellular, humoral and chronic rejection, graft-versus-host disease(GVHD), lymphoproliferative syndrome(PTLD), hematological alterations and death) are analyzed in patients who spleen was included as part of the intestinal graft, in splenectomized patients and those who preservate their native spleen
RESULTS103 transplants were performed26 Intestinal transplantation isolated, 22 liver-small bowel, 52 multivisceral and 3 modified multivisceral. 79% were first grafts, and 21% were retransplantation (27% third graft). Spleen was included as part of the graft in 11.7% patients, their native spleen was preserved in 50.5% and splenectomy was performed in 37.8%. Analyzing comorbidities, humoral rejection was infrequent; and it´s only present in patients with native spleen(4%), presenting positive antibodies without rejection in 17%, compared to 2.5% of splenectomized. Chronic rejection, itʼs 4 times more frequent in native spleen versus splenectomized(OR:4, CI:2-30). None of the patients with transplanted spleen presented chronic or humoral rejection. Cellular rejection is 2 times more frequent in native spleen instead patients with spleen transplanted(OR:2.2, CI:0.5-10.6) and itʼs 1.5 times more frequent in splenectomized(OR:1.5, CI:0.3-7.6). GVHD is 6 times more frequent in patients with transplanted spleen than in patients who preserve the spleen(OR:6, CI:2.2-13), followed by splenectomized(OR:2.2, CI:1.5-8.2). PTLD is 1.8 times more frequent in patients who preserve their spleen, compared to splenectomized(OR:1.8, CI:0.6-5.5), followed by patients with spleen transplanted(OR:1.4,CI:0.2-9.9). Haematological alterations are 79% more probably in patients with spleen transplanted (OR:3.8, CI:1-14) and 63% more probably in splenectomized versus those that preserve their native spleen(OR:1.7, CI:0.5-5.8). Death is 87% more probably in patients who include the spleen in the graft, compared to those who preserve their native spleen(OR:6.7, CI:1.8-24), followed by splenectomized in whom death is 87% more probably(OR:5,IC:2-13)
CONCLUSIONRejection is more frequent in patients with native spleen. Probably due to the confusional factor that supposes the type of transplant performed like the isolated intestinal transplant, not including the liver graft, and is well known the protective factor that this supposes for rejectionInclude spleen as part of the intestinal graft is a risk factor to develop PTLD, hematologic alterations and death. So the inclusion of the spleen as part of the intestinal/multivisceral graft is not recommendedPreserve native spleen is a risk factor to develop GVHD. However, it seems to be a protective factor against the development of other comorbidities, so itʼs recommended to preserve native spleen whenever is possible
