Experiencia de una unidad aerodigestiva pediátrica en Latinoamérica / Case management experience in a pediatric aerodigestive unit in Latin-America

Introducción: las unidades aerodigestivas proveen un cuidado complejo coordinado a pacientes pediátricos con condiciones congé-nitas o adquiridas de grados variables que afectan la vía respiratoria, el tracto gastrointestinal, la deglución y el crecimiento. La primera unidad aerodigestiva de Guatemala fue fundada en el año 2018 en un hospital privado, y por ser la única de su tipo en el país, no se tenían datos locales al respecto. Pacientes y métodos: se llevó a cabo un estudio retrospectivo cuantitativo; se analizó el total de casos reportados en la unidad aerodigestiva pediátrica desde el 1 de enero de 2018 hasta el 31 de mayo de 2019, se contó con un total de 69 casos. Resultados: el 79% de los pacientes presentaron más de una patología de diferente etiología. La indicación principal para realizar la triple endoscopia fue tos crónica. Los diagnósticos encontrados como comorbilidades con mayor frecuencia fueron bronquitis bacteriana recurrente y enfermedad por reflujo gastroesofágico. Conclusión: la triple endoscopia es útil tanto en el diagnóstico como en la toma de decisiones en el manejo de las patologías aerodigestivas pediátricas de alta complejidad. Es una herramienta útil que pone en evidencia que múltiples etiologías pueden contribuir a síntomas crónicos de la vía aérea, y que estas pueden pasarse por alto si se realiza un solo procedimiento.

Introduction: Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or ac-quired conditions affecting breathing, the gastrointestinal tract, swallowing, and growth. The first aerodigestive program in Guatemala was founded in the year 2018 in a private hospital and, since it's one of its kind in the country, there was no local data reported. Patients and methods: A total of 69 cases of children who were evaluated at the aerodigestive program from January 1st, 2018 to May 31st, 2019, were retrospectively analyzed in a quantitative study. Results:79% of patients presented more than one pathology from different etio-logy. Overall the main indication for the triple scope was chronic cough. The findings reported that recurrent bacterial bronchitis and gastroesophageal reflux were the most common comorbidities present in the same patient. Conclusion:The triple scope procedure is a useful investigative tool for patients with recalcitrant aero-digestive complaints. In particular, the triple scope can yield more than one specialty-specific diagnosis normally missed by one procedure.

Relationship between hepatic CTGF expression and routine blood tests at the time of liver transplantation for biliary atresia: hope or hype for a biomarker of hepatic fibrosis.

BACKGROUND: Progressive hepatic fibrosis (HF) is a prominent feature of biliary atresia (BA), the most common indication for liver transplantation (LT) in children. Despite its importance in BA, HF is not evaluated in routine patient care because the invasiveness of liver biopsy makes histologic monitoring of fibrosis unfeasible. Therefore, the identification of noninvasive markers to assess HF is desirable especially in children. PURPOSE: The main goal of this pilot project was to establish an investigational framework correlating hepatic expression of fibrogenic markers with routine blood tests in BA. METHODS: Using liver explants from patients with BA (n = 26), immune-expression of connective tissue growth factor (CTGF), a key fibrogenic cytokine was determined using horseradish-labeled antibodies. Expression intensities of lobular (L-CTGF) and portal (P-CTGF) CTGF were determined by using ImageJ software. These CTGF intensities were correlated with blood tests performed at the time of LT. Correlation coefficients were determined for each blood test variable versus mean L-CTGF and P-CTGF expression intensities. A P-value of less than 0.05 was considered statistically significant. RESULTS: All patients had end-stage liver disease and persistent cholestasis at the time of LT. Kendall tau (τ) rank correlation coefficient for L-CTGF and white blood cell (WBC) was inversed (-0.52; P ≤ 0.02). Similar but statistically nonsignificant inverse relationships were noted between L-CTGF and prothrombin time (PT) (-0.15; P ≤ 0.4), international normalized ratio (INR) (-0.14; P ≤ 0.5), and platelet count (-0.36; P ≤ 0.09). Inversed (τ) rank correlation coefficients were also evident between P-CTGF expression and gamma-glutamyl transpeptidase (GGT), PT, INR, and platelet count. Pearson correlation coefficients for combinational analysis of standardized total bilirubin (TB), alkaline phosphatase, GGT, and platelet count with L-CTGF (0.33; P = 0.3) and P-CTGF (0.06; P = 0.8), were not significant. Similar analysis for alanine aminotransferase, TB, and GGT combination (L-CTGF, 0.16; P = 0.5; P-CTGF -0.3; P = 0.2) as well as WBC, platelet count, and TB (L-CTGF: -0.36; P = 0.09; P-CTGF -0.33; P = 0.13) also revealed nonsignificant results. CONCLUSION: Hepatic expression of fibrogenic markers can be correlated with routinely performed blood tests in patients with BA. We document that although a trend of inverse relationship is noted, hepatic CTGF expression does not correlate well with routinely performed blood tests in advanced BA. Further work is required to determine more reliable ways of noninvasive diagnosis of HF.

Immunohistochemical localization of transforming growth factor ß-1 and its relationship with collagen expression in advanced liver fibrosis due to biliary atresia.

PURPOSE: Biliary atresia (BA) is the most common indication of liver transplantation in children. Pathogenesis of hepatic fibrosis, which is a prominent feature of BA, remains obscure. The purpose of this work was to determine the cellular sources of transforming growth factor beta-1 (TGFß1) and establish the relationship between TGFß1-producing cells and extracellular matrix producing myofibroblasts (MFBs) in advanced BA. METHODS: Trichrome staining and immunohistochemistry were carried out to determine the expression pattern of collagen and TGFß1 protein in explant liver specimens from patients with BA. The intensities of portal and lobular TGFß1 expressions were compared. Immunofluorescence technique was carried out to determine the relationship between α-smooth muscle actin (α-SMA)-positive-MFB and TGFß1-positve cells. RESULTS: Lobular TGFß1 protein expression was significantly higher than portal (89 ± 6 versus 10 ± 1 arbitrary units, P ≤ 0.05), whereas no difference was noted in livers used as control (10 ± 1.6 versus 19 ± 5 arbitrary units, P = 0.11). TGFß1 expression was more in the center of nodules versus MFB in surrounding fibrous septa. Contrary to TGFß1 expression, α1-SMA was mostly expressed in the portal structures and the adjacent fibrous septa enacting lobulation of the parenchyma. The results obtained by coimmunofluorescence staining showed no colocalization of α-SMA and TGFß1. CONCLUSIONS: TGFß1 protein expression is mostly localized to hepatocytes in advanced BA. These findings suggest a paracrine mechanisms of TGFß1-driven fibrogenesis in advanced BA.