Avaliação e suporte nutricional na criança com colestase / Nutrition assessment and support of children with cholestasis

A colestase acomete 65% dos pacientes pediátricos com hepatopatia, sendo responsável por várias consequências clínicas, como: retenção hepática de substâncias excretadas pela bile, lesão hepática progressiva, má-absorção intestinal de gorduras e vitaminas lipossolúveis, anorexia e esteatorreia. Há risco subestimado de desnutrição nesses pacientes, que está associada a grande morbidade. Para classificar os pacientes quanto ao estado nutricional, usam-se os índices de avaliação do crescimento. Porém, considerando a condição clínica do paciente, que pode incluir visceromegalias e ascite, os índices que utilizam o peso na análise podem ser imprecisos. Nesses casos, o uso das medidas de pregas cutâneas e da circunferência braquial leva a avaliações mais fidedignas. O paciente com colestase exige suporte nutricional para compensar a má-absorção e possível desnutrição. Esse suporte inclui: aporte calórico elevado,ingestão proteica em níveis que não se induza hiperamonemia, oferta de ácidos graxos majoritariamente de cadeia média, suplementação de vitaminas lipossolúveis (A,D, E e K) e alguns minerais. Adequado suporte nutricional pode evitar a progressão rápida da doença hepática, facilitar o processo de cicatrização, aumentar a função imunológica, além de prevenir várias consequências da deficiência de uma variedade de micro ou macronutrientes que pode ocorrer na colestase...

Cholestasis affects 65% of pediatric patients with liver disease and it is responsible for several clinical consequences such as liver retention of substances excreted in bile, progressive liver damage, intestinal malabsorption of fats and fat-soluble vitamins, anorexia, and steatorrhea. There is an underestimated risk of malnutrition in these patients, whichis associated with high morbidity. Indexes of growth evaluation are used to classify patients according to their nutritional status. However, considering the clinical condition of the patient, which may include increase of some organs?s size and ascites, the indices that use weight in the analysis may be inaccurate. In these cases, the use of measures of skinfold thickness and arm circumference leads to more reliable evaluations. The patient with cholestasis requires nutritional support to compensate the malabsorption and possible malnutrition. This support includes: caloric intake higher than usual, protein intake at levels which do not induce hyperammonemia, an offer of fatty acids predominantly mediumchain (absorbed independently from the action of micellar bile acids), supplementation of fat-soluble vitamins (A, D, E and K) and some minerals. An adequate nutritional support can avoid the fast progression of liver disease, facilitate the healing process, and enhance immune function, besides of preventing many consequences from the deficiency of a variety of micro or macronutrients which may happens in cholestasis...

Síndrome de Alagille experiencia clínica de catorce casos en Medellín, Colombia / Alagille syndrome, clinical experience of fourteen cases in Medellin, Colombia

Introducción: El Síndrome de Alagille corresponde a una alteración autosómica dominante con expresión variable. Se caracteriza por colestasis crónica con escasez de los conductos biliares interlobulares asociada a alteraciones cardiovasculares, oftálmicas, sistema esquelético, riñones y facies característica. Su distribución es mundial con frecuencia de 1 por cada 100000 nacidos vivos. Objetivo: Describir las características clínicas, la evolución y la sobrevida de catorce pacientes, con diagnóstico de Síndrome de Alagille atendidos en un período de 16 años en Medellín, Colombia. Materiales y métodos: Es un trabajo observacional descriptivo de reporte de casos de los hallazgos morfológicos y la evolución de los pacientes con diagnóstico de Síndrome de Alagille. Resultados: Grupo compuesto por ocho niños y seis niñas, con edades entre los dos meses y los diez años al momento de diagnóstico. El síndrome completo se presentó en 28%. Los hallazgos más frecuentes, estenosis valvular de la arteria pulmonar y la alteración vertebral se presentaron en el 79%. Tres pacientes 21%, fallecieron, uno de ellos después de recibir trasplante hepático. De los once sobrevivientes dos niñas fueron sometidas a trasplante y se encuentran en buenas condiciones. Los nueve restantes padecen hepatopatía colestásica crónica y reciben tratamiento médico. Conclusión: El Síndrome de Alagille se debe tener en cuenta en el diagnóstico de colestasis crónica infantil. Para establecer la distribución y frecuencia de esta enfermedad en nuestro país es necesario desarrollar investigaciones que idealmente incluyan el estudio de la mutación genética en los pacientes y su familia cercana.

Introduction: The Alagille Syndrome is an autosomic dominant disorder with variable expression. Chronic cholestasis, characteristic facial appearance and abnormalities heart, skeleton, eye, kydnes with hypoplasia of the biliary ducts. Initial description in France, with mundial distribution her frecuence is 1/100000. Objective: To describe the clinical characteristic and evolutions of fourteen patients with diagnosis Alagille Syndrome in Medellín Colombia. Materials and methods: Descriptive retrospective study with variables obtained from clinical records of patients with diagnosis Alagille Syndrome. Results: Eight boys and six girls. The age at diagnosis varied two months at nine years. Complete syndrome was present in 28%. The most frecuent alterations were valvular stenosis pulmonary artery and failure of anterior vertebral arch fusion (butterfly vertebrae) 79%. The clinical evolution was variable, death occurred in three patients 21%, one girl post liver transplantation. Nine children had chronic hepatopathy controlled with medical treatment and two girls had liver transplantation with satisfactory evolution. Conclusions: In Colombia, the poblational incidence is not defined it is necessary to know the distribution of syndrome at future study.

Protective effect of Jasonia montana against ethinylestradiol-induced cholestasis in rats

Estrogens, and particularly glucuronides such as ethinylestradiol [EE], have been shown to cause cholestasis in animal studies, by reducing bile acid uptake by hepatocytes. The aim of the present article is to investigate anticholestatic activity of the ethanolic extract of the aerial parts of Jasonia montana against liver cholestasis induced by EE in adult female rats in an attempt to understand its mechanism of action, which may pave the way for possible therapeutic applications. Subcutaneous administration of 100 micro g/kg b.w. ethinylestradiol to rats induced hepatocellular cholestasis with a significant decrease in serum cholesterol, bile acids and bilirubin levels as well as in hepatic superoxide dismutase [SOD], glutathione peroxidase [GPx], glutathione reductase [GR] activities and hepatic total, protein-bound and non-protein sulfhydryl groups. Also, treatment with EE produced significant increase in serum Pi-glutathione-s-transferase [Pi-GST], gamma glutamyl transpeptidase [gamma-GT] and alpha-glutathione-s-transferase [alpha-GST] activities as well as serum nitric oxide [NO] and tumor necrosis factor alpha [TNF-alpha] level and hepatic malondialdehyde [MDA] level as compare to control group. Oral administration of the aerial parts of ethanolic extract at a concentration of 150 mg/kg b.w. daily to rats treated with EE for 15 days showed a significant protection against-induced decrease in serum cholesterol, bile acids and bilirubin levels. The treatment also resulted in a significant increase in hepatic SOD, GPx and GR activities as well as hepatic total, protein-bound and non-protein sulfhydryl groups. In addition, the extract could inhibit serum Pi-GST, gamma-GT and alpha-GST activities as well as reduce serum TNF-alpha, NO and hepatic MDA as compare to ethinylestradiol treated rats. High content of flavonoids and phenolic compounds was found in ethanolic extract, which may be responsible for free radical activity. The results clearly suggest that the aerial parts of J, montana extract may effectively normalize the impaired antioxidant status in ethinylestradiol [EE]-cholestatic model. Thus the extract may have a therapeutic value in drug-induced biliary cholestasis as well as in hormonal therapy

Anticholestatic effect of picroliv, active hepatoprotective principle of Picrorhiza kurrooa, against carbon tetrachloride induced cholestasis.

Picroliv showed a dose (3-12 mg/kg, po for 7 days) dependent choleretic activity as evidenced by increase in bile flow and its contents (bile salts and bile acids). Significant anticholestatic activity was also observed against carbon tetrachloride induced cholestasis in conscious rat, anaesthetized guinea pig and cat. Picroliv was more active than the known hepatoprotective drug silymarin.