ABSTRACT
La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica
Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon
Subject(s)
Humans , Male , Child , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Lymphoma, Non-Hodgkin/pathology , Cholestasis/etiology , Jaundice, Obstructive/diagnosis , Jaundice, Obstructive/etiology , Jaundice, Obstructive/pathology , Pancreas , Syndrome , Cholestasis/diagnosisABSTRACT
La hidatidosis pancreática representa el 0,2-0,6 % de los casos, siendo la población pediátrica la de mayor riesgo. Las lesiones suelen localizarse en cabeza del páncreas (50-58 %); la localización en cuerpo y cola del páncreas se encuentra en el 24-34 % y el 19 %, respectivamente. Dada la posibilidad de complicaciones, suele realizarse tratamiento quirúrgico. Se sugiriere indicar albendazol antes y después del acto quirúrgico por los riesgos de ruptura y diseminación de los protoescólices. Se presenta el caso de una niña de 5 años de edad con dolor abdominal progresivo y lesión quística en páncreas compatible con hidatidosis en la ultrasonografía. En la tomografía computada se observa compresión de la vía biliar. La hemoaglutinación indirecta fue negativa. Presentó elevación de la bilirrubina total, con franco predominio de bilirrubina directa, y aumento de enzimas hepáticas. Se realizó laparotomía exploradora, colecistectomía y destechamiento del quiste. Evolucionó favorablemente, continuó con albendazol durante 3 meses luego de la cirugía.
Pancreatic echinococcosis accounts for 0.20.6% of cases, with the pediatric population being at a higher risk. Most commonly, pancreatic lesions occur in the head of the pancreas (5058%); and in the body and tail in 2434% and 19% of cases, respectively. Given the potential complications, surgery is usually performed. Albendazole is recommended before and after the surgery due to the risks for rupture and dissemination of protoscolices. Here we describe the case of a 5-year-old girl with progressive abdominal pain and cystic lesion in the pancreas compatible with echinococcosis in the ultrasound. The computed tomography showed bile duct compression. Indirect hemagglutination was negative. She had elevated total bilirubin, with a clear predominance of direct bilirubin, and high liver enzymes. Exploratory laparotomy, cholecystectomy, and unroofing of the cyst were performed. The patient had a favorable course and continued with albendazole for 3 months after the surgery.
Subject(s)
Humans , Female , Child, Preschool , Pancreatic Diseases/surgery , Pancreatic Diseases/complications , Pancreatic Diseases/diagnosis , Echinococcosis/surgery , Echinococcosis/complications , Echinococcosis/diagnosis , Pancreas , Albendazole/therapeutic use , AbdomenABSTRACT
Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an al- tered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical mani- festations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pedia- tric age is uncommon.
La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica.
Subject(s)
Cholestasis , Jaundice, Obstructive , Lymphoma, Non-Hodgkin , Male , Humans , Child , Cholestasis/diagnosis , Cholestasis/etiology , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Lymphoma, Non-Hodgkin/pathology , Jaundice, Obstructive/diagnosis , Jaundice, Obstructive/etiology , Jaundice, Obstructive/pathology , Pancreas , SyndromeABSTRACT
Pancreatic echinococcosis accounts for 0.2-0.6% of cases, with the pediatric population being at a higher risk. Most commonly, pancreatic lesions occur in the head of the pancreas (50-58%); and in the body and tail in 24-34% and 19% of cases, respectively. Given the potential complications, surgery is usually performed. Albendazole is recommended before and after the surgery due to the risks for rupture and dissemination of protoscolices. Here we describe the case of a 5-year-old girl with progressive abdominalpain and cystic lesion in the pancreas compatible with echinococcosis in the ultrasound. The computed tomography showed bile duct compression. Indirect hemagglutination was negative. She had elevated total bilirubin, with a clear predominance of direct bilirubin, and high liver enzymes. Exploratory laparotomy, cholecystectomy, and unroofing of the cyst were performed. The patient had a favorable course and continued with albendazole for 3 months after the surgery.
La hidatidosis pancreática representa el 0,2-0,6 % de los casos, siendo la población pediátrica la de mayor riesgo. Las lesiones suelen localizarse en cabeza del páncreas (50-58 %); la localización en cuerpo y cola del páncreas se encuentra en el 24-34 % y el 19 %, respectivamente. Dada la posibilidad de complicaciones, suele realizarse tratamiento quirúrgico. Se sugiriere indicar albendazol antes y después del acto quirúrgico por los riesgos de ruptura y diseminación de los protoescólices. Se presenta el caso de una niña de 5 años de edad con dolor abdominal progresivo y lesión quística en páncreas compatible con hidatidosis en la ultrasonografía. En la tomografía computada se observa compresión de la vía biliar. La hemoaglutinación indirecta fue negativa. Presentó elevación de la bilirrubina total, con franco predominio de bilirrubina directa, y aumento de enzimas hepáticas. Se realizó laparotomía exploradora, colecistectomía y destechamiento del quiste. Evolucionó favorablemente, continuó con albendazol durante 3 meses luego de la cirugía.
Subject(s)
Echinococcosis , Pancreatic Diseases , Female , Humans , Child , Child, Preschool , Albendazole/therapeutic use , Pancreatic Diseases/diagnosis , Pancreatic Diseases/surgery , Pancreatic Diseases/complications , Echinococcosis/diagnosis , Echinococcosis/surgery , Echinococcosis/complications , Abdomen , PancreasABSTRACT
INTRODUCTION: Cystic echinococcosis is endemic in Argentina. The standard pharmacological treatment for the disease is albendazole, but surgery is a common alternative. Even though primary infection occurs mainly in the pediatric population, the optimal therapeutic option in pediatrics is not clearly defined and few pediatric cohorts with cystic echinococcosis treated with albendazole have been described to date. OBJECTIVE: To describe therapeutic response to albendazole in a cohort of pediatric patients with abdominal cystic echinococcosis. POPULATION AND METHODS: Patients (0-18 years old) with abdominal cystic echinococcosis who were treated with albendazole between January 1998 and August 2013. Diagnosis of abdominal cystic echinococcosis was made by ultrasound. All patients received albendazole, 10-15 mg/kg/day. Epidemiological data, symptoms, number, location and outcome of the cysts, serology and treatment received were analyzed. The parameter used to assess treatment response was cyst changes evaluated by ultrasound follow up using the WHO-IWGE classification. RESULTS: A total of 28 patients (with 46 abdominal cysts) were included in the cohort. Mean age at enrolment was 9.4 years and mean duration of follow-up, 23.8 months. All patients resided in rural areas and had had contact with dogs. The asymptomatic form of the disease was the most common presentation. All patients received albendazole (mean duration: 142.5 days), with low incidence of adverse events. Albendazole had a positive effect on most of the cysts. Surgery was performed in 13 patients. CONCLUSION: Treatment with albendazole for uncomplicated cystic echinococcosis cysts is safe and effective, and can potentially reduce the need for surgical intervention.
Subject(s)
Albendazole/therapeutic use , Anthelmintics/therapeutic use , Echinococcosis/drug therapy , Adolescent , Animals , Argentina , Child , Child, Preschool , Echinococcosis/surgery , Female , Humans , Male , Retreatment , Retrospective Studies , Treatment OutcomeABSTRACT
Hydatidosis is a zoonosis of worldwide distribution caused by the parasite Echinococcus granulosus. Clinical manifestations include cyst formation, most commonly in the liver (67-89%) and lungs (10-15%). Renal localizations are rare. We report a case of renal hydatidosis in a five-year-old child treated in a tertiary pediatric hospital in Argentina. After the diagnosis was made, elective surgery was performed, which led to a rapid recovery.
Subject(s)
Echinococcosis/diagnosis , Kidney/parasitology , Albendazole/therapeutic use , Animals , Argentina , Child, Preschool , Cysts/parasitology , Echinococcosis/drug therapy , Echinococcosis/surgery , Echinococcus granulosus/isolation & purification , Eosinophilia/blood , Eosinophilia/diagnosis , Eosinophilia/parasitology , Hospitals, Pediatric , Humans , Kidney/diagnostic imaging , Liver/diagnostic imaging , Liver/parasitology , Lung/diagnostic imaging , Lung/parasitology , Male , Postoperative Complications/blood , Postoperative Complications/parasitology , UltrasonographyABSTRACT
Chronic granulomatous disease (CGD) is a primary immunodeficiency that affects the oxidative mechanism of microbial killing of phagocytic cells. The defect is characterized by a lack or severely reduced superoxide anion (O2-) production by phagocytes. Seventy percent of CGD cases are X-linked (X-CGD) and they are caused by mutations in the gene encoding for gp91(phox), one of the two subunits of the flavocytochrome b558 of the NADPH oxidase. We identified an abnormal transcript arising from a novel splice site mutation within the gene encoding gp91(phox), which suggested that the mutation affected normal mRNA splicing. Thus, the effect of this mutation leads to the complete absence of the flavocytochrome b558 in neutrophil membranes, which caused the biochemical phenotype X91 degrees-CGD in this family. These molecular findings help to explain the early onset and severe phenotype in this X-CGD kindred.
Subject(s)
Granulomatous Disease, Chronic/genetics , Membrane Glycoproteins/genetics , Mutation , NADPH Oxidases/genetics , Child , Cholangitis/etiology , Cholangitis/pathology , Humans , Liver Abscess/etiology , Liver Abscess/pathology , Male , NADPH Oxidase 2 , Neutrophils/metabolism , RNA Splice SitesABSTRACT
Se analizan retrospectivamente 22 pacientes portadores de necrosis pancreática. De los exámenes complementarios por imágenes, la tomografía computada es la más útil para el diagnóstico y evaluación de la extensión de la necrosis pancreática y peripancreática. Si bien la presente serie favorecería la indicación de necrosectomía y cierre primario del abdómen abierto, el número de pacientes y la imposibilidad ética de realizar estudios "randomizados", impiden conclusiones definitivas. La mortalidad global fue del 54% y las causas más importantes, fallas multiorgánicas y sepsis. Se estima que la alta mortalidad se debe a demora en el diagnóstico, debridamiento incompleto e insuficiente apoyo nutricional.
Subject(s)
Humans , Adult , Middle Aged , Male , Female , Necrosis , Pancreatitis/diagnosis , Acute Disease , C-Reactive Protein , Ileostomy , Pancreatitis/epidemiology , Postoperative Complications , Prognosis , ReoperationABSTRACT
Se analizan retrospectivamente 22 pacientes portadores de necrosis pancreática. De los exámenes complementarios por imágenes, la tomografía computada es la más útil para el diagnóstico y evaluación de la extensión de la necrosis pancreática y peripancreática. Si bien la presente serie favorecería la indicación de necrosectomía y cierre primario del abdómen abierto, el número de pacientes y la imposibilidad ética de realizar estudios "randomizados", impiden conclusiones definitivas. La mortalidad global fue del 54% y las causas más importantes, fallas multiorgánicas y sepsis. Se estima que la alta mortalidad se debe a demora en el diagnóstico, debridamiento incompleto e insuficiente apoyo nutricional. (AU)
