Enfrentamiento de pacientes pediátricos con várices esofágicas / Management of pediatric patients with esophageal varices

Background: There is a paucity of good quality research about the diagnosis of esophageal varices and the prophylaxis and treatment of variceal bleeding in pediatric patients with portal hypertension There is little consensus and practically no evidence-based approach about the management of these patients. Aim: To describe the behavior and preferences of pediatric gastroenterologists in Chile in the management of portal hypertension in children. Material and Methods: An online survey was sent to Chilean pediatric gastroenterologists, with questions evaluating the physicians’ approaches to screening of esophageal varices in children with portal hypertension, and their preferred methods of prophylaxis and initial management of variceal bleeding. Results: Thirty five of 69 contacted physicians answered the survey (51%). Twenty nine pediatric gastroenterologists (83%) screen for esophageal varices in patients with clinical evidence of portal hypertension, and 12 (34%) in every patient with chronic liver disease. Twenty eight respondents (80%) use primary prophylaxis, mainly beta blockers. Octreotide, proton pump inhibitors and endoscopy are the most common practices in the initial management of an esophageal varix bleed. The methods mostly used as secondary prophylaxis are band ligation and beta blockers. In the case of recurrent hemorrhage, besides band ligation, management with Transjugular Intrahepatic Portosystemic Shunt (TIPS) and hepatic transplantation are more likely. Conclusions: Even though most pediatric gastroenterologists in this survey are inclined to offer endoscopic screening of esophageal varices and prophylaxis to patients with portal hypertension, this is not a universal behavior. There are different approaches mainly in the election of secondary prophylaxis and the initial management of variceal bleeding.

Profilaxis primaria de várices esofágicas en pediatría / Primary prophylactic treatment of esophageal varices in pediatrics

Children with chronic liver disease or portal vein thrombosis frequently develop portal hypertension. The main complication of the portal hypertension are the development of esophageal varices who are at risk of variceal bleeding, which is associated with significant morbidity and mortality. Guidelines for adults with portal hypertension recommend performing esophagogastroduodenoscopy to identify those with varices that may benefit from prophylactic therapy, however there are no consensus in the pediatric age. This revision includes the incidence, diagnostic and the evidence for primary prophylactic treatment of the esophageal varices in children.

Pacientes pediátricos con daño hepático crónico o con trombosis de la vena porta frecuentemente desarrollan hipertensión portal. La principal complicación de la hipertensión portal es el desarrollo de várices esofágicas que conlleva riesgo de hemorragia variceal, la cual se asocia a significativa morbimortalidad. Las guías clínicas para adultos recomiendan realizar, en pacientes cirróticos, una endoscopia como screening para el diagnóstico de várices esofágicas y su tratamiento profiláctico, sin embargo, en pacientes pediátricos no existe consenso. En esta revisión se actualiza la incidencia, diagnóstico y evidencia del tratamiento profiláctico primario de várices esofágicas en pediatría.

Curvas manométricas rectales en niños: estudio promano fase I / Rectal mamometric curves in children: phase I study

Aim of this study: To evaluate the role of anorectal manometry (ARM) and determine normal ARM's parameters in a group of children referred for evaluation of defecation disorders. Patients and Methods: A retrospective review of pediatric ARMs performed over an 8-year period. Results: Records from 789 children (52.6 percent male) were reviewed. Patients were classified in four groups according to the main ARM's findings in: "Control group" (CL), "Probable megarectum group" (PMG), "Abnormalities of intrinsic innervation group" (All) and "Abnormalities of extrinsic innervation group" (AET). 79 percent of them had been referred for evaluation of chronic constipation and 10 percent because of suspicion of abnormalities of intrinsic innervation. In 94 and 83 percent of them respectively, the ARM ruled out organic causes. ARM's findings distribution was: CL (48.0 percent), PMG (42.6 percent), All (7.5 percent) and AEI (1.5 percent). CL and PMG showed age-dependent differences in manometric parameters. Differences in manometric parameters between CL, PMG, API and AEI were found. Conclusions: Four distinctive patterns were described in 789 patients referred for evaluation of their presumed defecation disorder. The main indication of ARM was study of chronic constipation, where organic problems were confirmed only in a few of them.

Propósito del estudio: Evaluar el rol de la manometría rectoanal (MRA) y establecer valores de normalidad en un grupo de niños referidos por desórdenes de defecación. Pacientes y Métodos: Revisión retrospectiva de MRA efectuadas durante un período de 8 años. Resultados: Se analizaron los resultados obtenidos de 789 niños (52,6 por ciento hombres). Éstos se clasificaron en cuatro grupos según resultado de la MRA como "Grupo control" (CL), "Grupo con probable megarrecto" (PMG), "Grupo con anormalidades de la inervación intrínseca" (All) y "Grupo con anormalidades de la inervación extrínseca" (ALE). Setenta y nueve por ciento de los pacientes fueron referidos para evaluación de constipación crónica y 10 por ciento por sospecha de anormalidades de inervación intrínseca; en 94 por ciento y 83 por ciento de ellos respectivamente, la MRA descartó causas orgánicas. Los niños se distribuyeron en: CL (48,0 por ciento), PMG (42,6 por ciento), All (7,5 por ciento) y AIE (1,5 por ciento). El grupo CL y PMG mostraron diferencias edad-dependiente en algunos parámetros manométricos. Además se encontró diferencias en parámetros manométricos entre CL, PMG, All y AIE. Conclusiones: La principal indicación de MRA fue para estudio de constipación crónica, siendo las alteraciones orgánicas confirmadas sólo en un bajo porcentaje. Se describió cuatro patrones diferentes en 789 pacientes referidos para evaluación de dificultades en la defecación.

Síndrome hemolítico urémico en Chile: presentación clínica, evolución y factores pronósticos / Hemolytic-uremic syndrome in Chile: clinical features, evolution and prognostic factors

Background: Hemolytic-uremic syndrome (HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Aim: To describe the characteñstics ofpatients with the diagnosis ofHUS in Chile, and to identify the most reliable early predictors oímorbidity and moñality. Material and methods: The clinical records ofpatients with HUS aged less than 15 years, attended between January 1990 and December 2003 in 15 hospitals, were reviewed. Demographic, clinical, biochemical, hematological parameters, morbidity and mortality were analyzed. Results: A cohort of 587 patients aged 2 to 8 years, 48 percent males, was analyzed. Ninety two percent had diarrhea. At the moment of diagnosis, anuria was observed in 39 percent of the patients, hypertension in 45 percent and seizures in 17 percent. Forty two percent required renal replacement therapy (RRT) and perítoneal dialysis was used in the majoríty of cases (78 percent). The most frequently isolated etiological agentwas Escherichia coli. Mortality rate was 2.9 percent in the acute phase of the disease and there was a positive correlation between mortality and anuria, seizures, white blood cell count (WCC) >20.000/mm³ and requirements of renal replacement therapy (p <0.05). Twelve percent of patients evolved to chronic renal failure and the risk factors during the acute phase were the need for renal replacement therapy, anuria, WCC >20.000/mm³, seizures and hypertension. Conclusions: The present study emphasizes important clinical and epidemiological aspeets ofHUSin a Chilean pediatricpopulation.

Evaluación de los índices de monitoreo de pH esofágico y su relación con indicaciones clínicas: MePeache Fase I / Evaluation of the esophageal pH monitoring and its relation with clinical indications: MePeache Phase I

Objectives: Establish mean values and normality range for pH metric parameters, different to reflux index (RI), in esophageal pH monitoring (EpHm) according to new RI cut- off values; besides, correlate them with clinical diagnosis. Method: Retrospective study of EpHm parameters including RI, number and average length of episodes (DPE), number of episodes > 5 minutes and duration of the longest episode. 153 patients were classified according to age (Group I < 1 year and Group II > 1 year) and RI cut-off values: Normal group (NL) with physiological range (RI < 12 percent GI and < 6 percent GII) and Pathological group (PT) when RI was higher. Results: The most frequent EpHm indications were gastroesophageal reflux (GER) study (41.7 percent), airway diseases (19.9 percent) and otolaryngologic diseases (17.2 percent). There was no correlation between EpHm parameters in GI (n = 53) when analyzed by gender, referring physician or EpHm indication. In GII (n = 100), the RI for children referred by pediatricians was higher than the one for those referred by gastroenterologists in Group NL (p = 0.002). There were differences in all pH metric parameters according to NL and PT groups, except DPE. 84.9 percent and 77 percent of the EpHms in GI and GII respectively were normal. Conclusions: The EpHm parameters allow the division in NL and PT groups according to new RI cut-off values. There is a weak correlation between clinical suspicion and pHmetric diagnosis, sugesting the necessity to reevaluate EpHm indications.

Objetivos: Establecer valores promedio y rangos de normalidad de parámetros pHmétricos diferentes al índice de Reflujo (IR) en monitoreos de pH esofágico (MpHe), según nuevos valores de IR y correlacionarlos con el diagnóstico clínico. Pacientes y Método: Estudio retrospectivo de parámetros de MpHe incluyendo IR, n° y duración de episodios (DPE), n° episodios > 5 min y duración episodio mayor. Se reagrupó a los 153 pacientes según edad (Grupo I < 1 año y Grupo II > 1 año) y según IR como: Grupo Normal (NL) a aquéllos con IR en rango fisiológico (IR < 12 por ciento GI y < 6 por ciento GII) y Grupo Patológico (PT), cuando el IR superaba estos valores. Resultados: Las indicaciones más frecuentes fueron estudio de RGE (41,7 por ciento), patología de vías aéreas (19,9 por ciento) y otorrinolaringológicas (17,2 por ciento). Al comparar MpHes en GI (n = 53) no se encontraron diferencias significativas según género, médico referente o indicación. En el Grupo NL de GII (n = 100) se encontró un IR en niños referidos por pediatras mayor al de aquellos referidos por gastroenterólogos (p = 0,002). Según grupos NL y PT, todos los parámetros pHmétricos resultaron significativos (p < 0,005), excepto la DPE. 84,9 por ciento y 77 por ciento de los MpHes en GI y GII, respectivamente fueron normales. Conclusión: Los índices de MpHe permiten separar los grupos NL y PT bajo los nuevos puntos de corte del IR. Existe escaso correlato entre sospecha clínica y diagnóstico pHmétrico lo que sugiere la necesidad de reevaluar sus indicaciones clínicas.

Evaluación prospectiva de la seguridad y tolerancia de los procedimientos colonoscópicos en pacientes pediátricos / Prospective evaluation of safety and tolerance of colonoscopy in children

Background: Colonoscopy is a well established diagnostic and therapeutic procedure in pediatrics. Aim: To evaluate colon preparation alternatives for colonoscopy or sigmoidoscopy, type of sedation, clinical indications and findings. Patients and methods: Prospective study of 123 children referred for colonoscopy. Demographic data, type of colon preparation, sedation, type of endoscope and endoscopic results were obtained. The following day, a phone interview was carried out inquiring about duration, quality and adverse effects of the sedation and procedure. Results: Seventy one boys (58%) and 52 girls (42%) with a mean age of 6.7±4.4 years, were recruited. The main indication was lower gastrointestinal bleeding (71%). The different colon preparations produced elimination of clear liquid stools in 50%, non transparent liquid in 23%, semi liquid in 22% and solid in 6% of the patients. Most common side effects were abdominal distension (20%) and nausea (16.8%). The most commonly used drugs were midazolam (76%) and demerol (43%). The average duration of the procedure was 18.3 minutes (range: 4-50). The most common findings were rectal polyps (18.7%) and hemorrhagic colitis (14.6%). In 77% of cases, the sedation was considered very good or good. Colon visualization was described as very good (51%) or good (36%). Seventy three percent of children had complete amnesia. The most common adverse effect was vomiting (7.5%). Conclusion: Lower endoscopies are feasible procedures to carry out in children, in an ambulatory basis, with intravenous sedation and minimum adverse effects.

Síndrome hemolítico urémico: Experiencia de un centro pediátrico / Hemolytic uremic syndrome: The experience of a pediatric center

Background:Hemolytic uremic syndrome (HUS) is one of the main causes of acute renal failure in the Chilean pediatric population. Aim: To report the features of patients with HUS, admitted to the pediatric ward of a clinical hospital. Material and methods: Retrospective review of medical records of patients admitted with the diagnosis of HUS between 1995 and 2002. Results: During the period, 58 patients were admitted with the diagnosis of HUS but only 43 (age range 1 month to 6 years, 22 females) had complete medical records for review. Ninety five percent presented with prodromic diarrhea, mainly dysenteric. Antibiotics were administered to 70%, in the previous days. Acute renal replacement, mainly peritoneal dialysis, was required in 40%. The clinical signs and laboratory parameters that correlated better with the indication for dialysis were anuria, hypertension, initial and permanently high serum creatinine and blood urea nitrogen. Four patients with blood urea nitrogen over 100 mg/dl but without anuria or hyperkalemia, were treated conservatively, and experienced an uneventful course (permissive azotemia). Hospital stay was almost 3 times greater in dialyzed than in non dialyzed children. No deaths related to HUS were reported in the study period. In an average follow up of 54 months, 11.6% of the patients developed chronic renal failure of diverse magnitude. Conclusions: Despite the fact that our study group behaved clinically similar to published HUS patients in other series, no mortality was observed in a retrospective analysis of patients with this disease.

Síndrome hemolítico urémico en Santiago de Chile: evolución de la función renal y factores pronósticos / Hemolytic uremic syndrome (HUS), prognostic factors and follow up of renal function, in Santiago, Chile

Introducción: El Síndrome Hemolítico Urémico (SHU) se caracteriza por falla renal aguda, anemia hemolítica microangiopática y trombocitopenia; es la causa más frecuente de insuficiencia renal aguda en la infancia. Objetivo: a) Describir las características actuales del cuadro inicial de SHU en nuestro medio y comparar con lo descrito anteriormente; b) Describir la evolución a 1 año plazo y evaluar posibles factores pronósticos de función renal. Sujetos y Métodos: Se estudiaron variables demográficas, presentación clínica, exámenes bioquímicos y hematológicos, en 374 pacientes con SHU diagnosticados entre Enero 1990 a Diciempe 2002 en 9 hospitales de la Región Metropolitana; se evaluó además función renal al año de seguimiento en una muestra de 213 pacientes y se identificaron factores pronósticos de insuficiencia renal crónica y mortalidad utilizando el análisis de regresión logística. Resultados: Se analizaron 374 pacientes, 50,5 por ciento mujeres, 65,5 por ciento de la Región Metropolitana, edad promedio 1,5 ± 1,4 años (0,2 a 8); 91 por ciento presentó diarrea, 31 por ciento ocurrió en verano, al ingreso 57 por ciento presentó anuria, 43,3 por ciento hipertensión arterial y convulsiones 23 por ciento. Al alta 28 por ciento persistía hipertenso. Las terapias de sustitución renal utilizadas fueron: diálisis peritoneal (50 por ciento), hemodiafiltración (6 por ciento) y hemodiálisis (3 por ciento); recibió plasmaféresis 1 por ciento. Se aisló agente etiológico en 17 por ciento, siendo en 69 por ciento E. coli enterohemorrágica. La mortalidad fue de 2,7 por ciento, siendo la causa principal la falla orgánica múltiple. En el seguimiento al año: 80 por ciento mantuvo función renal normal, 14 por ciento presento deterioro de la función renal, 6 por ciento proteinuria y 4 por ciento hipertensión. Se encontró significativo como factor pronóstico de daño renal: hipertensión arterial (p < 0,0001), necesidad de peritoneodiálisis y hemodiálisis (p: 0,001, p: 0,0015 respectivamente), anuria (p: 0,005) y convulsiones (p: 0,01). Se correlacionó con mortalidad en la etapa aguda: convulsiones, requerimiento de hemodiafiltración y plasmaféresis, (p < 0,0001, p: 0,0001 y p < 0,0001 respectivamente).