Caroli's Syndrome: An Early Presentation.

Fibropolycystic liver disorders (FLD) arise from abnormal development of the ductal plate and are classified according to the size of the affected hepatobiliary duct. Congenital hepatic fibrosis (CHF) has small duct involvement characterized by a variable degree of periportal fibrosis and hyperplasia without affecting the liver's architecture. Caroli's disease (CD) is a rare autosomal recessive disorder with a prevalence of one case per 1,000,000 people and is characterized by cystic dilation of large intrahepatic ducts. When the disease presents with congenital hepatic fibrosis, it is referred to as Caroli's syndrome (CS). Patients are usually diagnosed around the age of 20 with episodes of cholangitis, portal hypertension or hepatomegaly. We present the case of a two-year-old male with a previous history of autosomal recessive polycystic kidney disease (ARPKD) who presented to the emergency room with variceal bleeding secondary to portal hypertension. The physical examination showed an acutely ill-looking boy, with evident paleness and distended abdomen. Past medical history was negative for previous gastrointestinal bleeding or episodes of cholangitis. An upper gastrointestinal endoscopy was performed, showing esophageal varices secondary to portal hypertension. Imaging studies revealed hepatosplenomegaly, alterations in liver echogenicity, and dilated saccular bile ducts affecting both liver lobes without observing any apparent obstruction, highly suggestive of CD. A liver biopsy revealed nodular liver tissue with marked fibrosis between nodules, which confirmed the presence of CHF. Both kidneys were increased in size, hyperechoic and with loss of corticomedullary differentiation. FLD commonly present with coexisting hepatobiliary and renal alterations. Therefore, starting at the time of initial diagnosis, all patients with ARPKD should be evaluated to detect liver abnormalities due to the high association. Despite the rarity of CS, especially in early childhood, the association between ARPKD and FLD is well documented. So if this clinical presentation arises, CS should be suspected.

Estandarización de ELISA IgM e IgA para el inmunodiagnóstico de la triquinosis humana / Standarization of ELISA IgM and IgA for inmunodiagnosis of human trichinosis

An ELISA test for trichinosis using as antigen a larvae soluble fraction from trichinella spiralis was carried out for the detection of IgM and IgA specific antibodies in 45 serum samples from patients confirmed or suspected to have trichinosis by strong clinical and epidemiological evidences. All the patients had positive serology detected by precipitin test, bentonite floculation test, indirect hemagglutination tes and ELISA IgG test. The cut off value was determined using two criteria. Criterion A was determined in each plate, using three positive controls and two negative ones; the average of the negative controls and the weakest positive control, muliplied by a 1,2 factor was, considered the cut off value. Criterion B was determinated using the average plus three standard deviations from 64 apparently halthy persons serum samples. In both cases, three serum dilutions (1:10, 1:100 and 1:500) were used. The sensitivity of ELISA IgM was 100,0, 93,3 and 82,2 percent using serum dilution of 1:10, 1:100 and 1:500 respectively (criterion A) and 100,0, 97,8 and 95,6 percent for the same dilutions (criterion B), whereas the values for ELISA IgA were: 100,0 91,1 and 86,7 percent (criterion A) and 100,0 100,0 and 91,1 percent (criterion B). In order to find out the specificity of ELISA IgM and ELISA IgA, additional 118 serum samples from individuals with other parasitoses, such as cysticercoss (18) hydatidosis (39), fascioliasis (12), toxocariasis (30), Chagaïs disease (12) and individuals with non specif eosinophilia (7), were also tested. ELISA IgM presentes a specificity of 92,3, 93,4 and 97,3 percent (criterion A) and 96,2, 97,8 and 97,8 percent (criterion B) whereas the results for ELISA IgA were 97,8, 98,9 and 99,4 percent (criterion A) and 98,4 percent for the 1:10 and 1:100 dilutions and 100,0 percent for the 1:500 dilution (criterion B). The positive predictive values of ELISA IgM were 76,3, 77,8 and 88,1 percent (criterion A) and 86,5, 91,7 and 91,5 percent (criterion B) whereas the negative ones were 100,0, 98,3 and 95,7 percent (criterion A) and 100,0, 99,4 and 98,9 percent (criterion B). The positive predictive values of ELISA IgA were 91,8, 95,3 and 97,5 percent (criterion A) and 93,8, 93,8 and 100,0 percent (criterion B) whereas the negatives ones were: 100,0, 97,9 and 96,8 percent (criterion A) and 100,0, 100,0 and 97,8 percent (criterion B). The use of ELISA IgM and ELISA IgA in the inmunodiagnosis of trichinosis is discussed

Esquemas del tratamiento para pacientes con labio y/o paladar fisurado y análisis de casos de pacientes del hospital Lorencita Villegas de Santos / Schemes of treatment for patient sustained fissured lip or palate and cases analysis of patients of the Hospital Lorencita Villegas de Santos

El labio y /o paladar fisurado, es la más común malformación congénita de cabeza y cuello. Su etiología es multifactorial; está determinada por predisposición genética, más factores ambientales. El labio fisurado se presenta en uno de cada 900 nacimientos vivos, afectando varones en mayor proporción; el paladar fisurado afecta uno de cada 2000 nacimientos y las niñas se ven mas afectadas. En la mayoria de los casos revisados durante el desarrollo de ésta investigación, se presentó la combinación de labio y paladar fisurado. Los factores exógenos, incluídos el uso de insecticidas, Fenitoína o Fármacos para el tratamiento del cáncer durante el embarazo, han sido incluídos en la etiología de fisuras faciales. Algunos casos de labio y paladar fisurado, han sido asociados con sindromes congénitos que afectan otras partes del cuerpo. La fisura del labio y/o paladar, esta establecida dentro de los 5 a 8 primeras semanas de vida intrauterina. En general estos defectos parecen tener causas y efectos ambientales mixtos. Las hendiduras del labio y proceso alveolar que se continuan a través del paladar, suelen transmitirse a través de la primera generación. Pueden producirse por falta de unión de procesos maxilares con el proceso nasal, o por penetración incompleta del mesodermo dentro de las membranas epiteliales del proceso nasal medio, del proceso nasal lateral y de los procesos maxilares. Los pacientes con labio y/o paladar fisurado requeriran supervisión médica desde el nacimiento y el manejo por parte de un equipo multidisciplinario que incluye cirujanos plásticos, ortodoncista, cirujano oral, foniatras y psicólogos entre otros. El manejo por parte de un equipo conduce no solo al mejoramiento de cada paciente individualmente sino que brinda una perspectiva de manejo adecuado de todos los pacientes que presentan esta amlformación. Las técnicas modernas para la reparación de labio fisurado estiman que la deformidad afecta no solo la piel sino el músculo orbicular y la maxila subyacente la cual tiende a ser hipoplásica. Como es sabido que todas la técnicas que existen para el tratamiento de estos pacientes son numerosas; podriamos deciacion medica