Pediatric liver transplantation: ten years of experience in a multicentric program in Chile.

Liver transplantation is the only treatment for patients with terminal acute and chronic diseases. Liver transplantation was started in Chile in 1985; our pediatric program began in 1993. The aim of this paper work was to present our experience from 1993 through 2004. One hundred and thirty two orthotopic liver transplants (OLT) were performed in children of mean age 5 years and median age 4 years (8 months to 15 years). The most frequent indications were biliary atresia, (43.1%) and acute liver failure (ALF; 20.4%), whose frequent cause was unknown but viral hepatitis A was the second one. A complete liver was transplanted in 59 patients, reduced in 39, split in one, and as an auxiliary liver in another one. Living related liver transplantation was performed in 32 cases (24.2%), of which thirty included segments II and III, and two, a right liver. A terminal arterial anastomosis was performed in 102 (77.2%) recipients and a graft interposition in 32 patients (24.2%). In 16 cases, biliary reconstruction was performed through an enterobiliary anastomosis. Immunosuppression included cyclosporine (Neoral), steroids, and azathioprine with conversion to tacrolimus (Prograf) as indicated. Rejection episodes, which were always biopsy-proven, were treated either with methylprednisolone or with antibodies. Biliary complications were the most frequent (21.4%) and the second cause was vascular complications (13%). Sixty-six patients suffered an acute rejection episode. Actuarial graft survival was 81.3% at 1 year and 72% at 5 years, while actuarial graft survival for ALF was 75.9% at 1 year and 67.8% at 5 years. Our results are comparable to those reported by most international groups.

Living related liver transplantation. Why this option has been discarded in a pediatric liver transplant program in Chile.

Living related living transplantation (LRLT) has opened new possibilities for planning transplantation in better conditions for children with emergency situations and chronic liver diseases. Since we began the LRLT program in 1999, we have performed 57 pediatric liver transplants, 17 (29.8%) using living related donors (LRD). The aim of this study was to analyze the reasons why LRD were discarded as a therapeutic option. All pediatric patients were prospectively included in our Microsoft Excel database that was reviewed for obtaining information about causes why the LRLT could not be done. LRLT was proposed in 28 cases and performed in 17 (60.7%). The reasons for LRD rejection were: parent's fear of surgical complications in four cases; drug abuse in two; a mother without family support; medical reasons in two; and only one, due to anatomical reasons and in one case, cadaveric graft transplantation was performed while completing the father's evaluation. From these eleven cases, the indications for liver transplant were acute liver failure (ALF) in seven, biliary atresia in three, and Alagille syndrome in one. Nine were transplanted with cadaveric organs, but two patients with ALF died awaiting a liver. Efforts should be made to clarify the advantages and the disadvantages of LRD in each case, allowing parents to make a free, well-informed decision.

Outcomes of orthotopic liver transplantation in Chile.

Our liver transplant program was started in 1993 in a private clinic and a public hospital. Thereafter, a rapid increase in adults and pediatric candidates for this therapeutic option lead to this analysis of results in 165 orthotopic liver transplants (OLT) in 143 patients between November 1993 and December 2002. Seventy-four OLT were performed in 66 adult patients and 91 in the pediatric group. Liver grafts came from cadaveric donors in 145 cases (74 adults and 71 children). The technique of living-related donor was utilized in 20 pediatric cases. Main indications for OLT in the adult group were HCV cirrhosis, primary biliary cirrhosis; biliary atresia and acute liver failure were the indications in pediatric patients. Retransplantation was needed for 23 patients, including 9 adults and 14 children. The most frequent causes of death were sepsis, graft primary nonfunction, and vascular complications. Actuarial survivals at 1 and 5 years were 80.7% and 72.6% for the adult group and 82% and 74.8% for the pediatric group, respectively. Our results are comparable to those published by large, experienced, international centers, with much better financial support.

Epidemiology and results of liver transplantation for acute liver failure in Chile.

Acute liver failure (ALF) is a severe, life-threatening condition associated with a high mortality rate. The objective of this study is to present the experience of a Chilean liver transplant program with orthotopic liver transplantation (OLT) for ALF. All patients with the diagnosis of ALF evaluated in our program between January 1995 and May 2003 were included in the analyses of etiology and outcomes. Candidates for OLT activated on a national waiting list were transplanted with cadaveric or living-related donor (LRD) organs. Twenty-seven patients age 1 to 19 years (median, 7.4 years) were transplanted at a median weight of 30.7 kg including 17 cadaveric and 10 with LRD livers. Most frequent etiologies were hepatitis A in 10 cases (37%) and unknown in 12 (48.1%). One donor experienced superficial phlebitis. Four patients were retransplanted (14.8%). Twenty patients are alive with 1- and 5-year survival rates of 74.1% At a median follow up of 34 months (range = 2 to 120). Seven patients died due to sepsis, multiorganic failure, graft primary nonfunction, intracranial hemorrhage, and intraoperative cardiac arrest. This experience revealed results comparable to international reports, allowing survival of patients destined to die.

[Eosinophilic colitis caused by allergy to cow's milk protein]. / Colitis eosinofílica por alergia a proteína de leche de vaca.

Eosinophilic colitis is one of the clinical manifestations of allergy to cow's milk during the first year of life. We report a series of 9 infants who, under 9 months of age and while clinically well, presented rectal bleeding of variable magnitude, with or without diarrhea, shortly after a cow's milk-based formula was initiated (n = 6); yet, 3 cases received only breast feeding. Bleeding disappeared in all patients after milk withdrawal from the diet. Challenges were planned after 12 months of treatment; three patients have not yet reached this moment, 3 had a negative challenge at 12, 18 and 28 months of age and are on a complete diet, and 3 are still on cow's milk free diet because ingestion of milk at 12, 18 and 25 months still induced rectal bleeding. This series of patients gathered in 3 years, follows the trend reported in many countries that there is a relative increase of patients diagnosed with allergy conditions early in life.

[Experience of a single center in liver transplantation in adults and children]. / Experiencia de un centro en trasplante hepático en adultos y niños.

Orthotopic liver transplantation (THO) is the treatment of choice for a variety of liver diseases. The national experience before 1993 has been scarce. In November 1993 we started our experience in THO at Clínica Las Condes, as part of a multiorgan transplant program (liver, kidney, pancreas). Until January 1995 we have performed 14 THO in 13 recipients (one retransplantation), of which 5 were in pediatric cases. The recipients range of age fluctuated between 1 and 61 years. In two pediatric cases a liver allograft reduction was performed. Six recipients (46%) required treatment for acute cellular rejection. One recipient had an hepatic artery thrombosis and had to be retransplanted. There was no operative mortality up to 30 days in cases of primary liver transplants. The retransplanted adult recipient, and another pediatric recipient that died from a late recurrence of a hepatoblastoma, accounted for the mortality of this experience. After an average follow up of 10 months, the actual patient survival in 85%. Of the 11 surviving recipients, 9 are in excellent conditions with a very good quality of life. This experience shows that an active liver transplant program in our country can be successful in obtaining results comparable to those published by very experienced foreign transplant centers.

[Celiac disease in adolescents]. / Enfermedad celíaca en la adolescencia.

Nutritional status, sexual maturation and intestinal absorption were assessed in 16 adolescent celiac patients (12 females) at ages 12 and 18 year. Weight/age, height/age and weight/height were 75.5% (51.6-94.8), 90.3% (76.8-104.6) and 99.1% (76.3-112.9) at 12 years and 85.6% (64.0-105.2), 93.8% (85.9-101.2) and 110% (76.5-121.9) at 18 years of age, respectively. Sexual maturation (Tanner) was at stage I in 31%, and at stage II in 43.7% of patients at age 12 years and it had reached maturity in 75% of them at 18 years. Menarche occurred between 12 and 14 years of age, except in 3 patients in whom it was retarded. Mean serum carotene levels were 125 and 108.5 ug/dl at ages 12 and 18 years respectively. Delayed weight and height progress in this particular group of patients might be explained by low socio-economic conditions, late diagnosis, and poor adherence to gluten-free diet.

[Ulcerative colitis]. / Colitis ulcerosa.

Ulcerative colitis in children has been seldom described in Chile. The cases of a 14 year old girl and a 10 year old boy with this disease are presented. Both had diarrhea for more than two months--which was continuous in the first case and intermittent in the other one--, bloody stools, weight loss, anemia and abdominal pain. Bacteriological and parasitological examination of stools were negative. Diagnosis of ulcerative colitis was based on barium enema, which showed mucosal ulceration and loss of the normal claustral pattern, rectosigmoidoscopy, that revealed hyperemia, friability and erosions of the corresponding segments of intestinal mucosa, and on histological examination of multiple mucosal biopsies, which disclosed crypt abscess, distorted crypt pattern, inflammation of the lamina propria and decreased number of goblet cells. Both cases were treated with salazosulfapyridine with satisfactory response.

Hepatitis neonatal por virus rubeola. / Neonatal hepatitis due to rubella virus

La ictericia colestasica del primer trimestre representa un problema diagnostico, ya que no siempre corresponde a malformaciones de la via biliar, sino que tambien puede encontrarse en enfermedades hepatocelulares, de causa infecciosa, metabolica o desconocida. Se presenta el caso de una nina de 1 mes de edad con ictericia de tipo colestasico y hepatoesplenomegalia, en que los estudios de laboratorio e histologico demostraron la existencia de hepatitis neonatal por virus rubeola

Reflujo gastroesofagico: evolucion clinica y radiologica. / Gastroesophageal reflux: clinical and radiological course

Se evaluaron 90 ninos con RGE clinico y radiologico, cuyas edades en la primera consulta fueron: menos de 12 meses: 62; 12 -24 meses: 18; y mayor de 24 meses:10.Consultaron por vomitos el 71.1% y por sintomas broncopulmonares 24.5%. Con el tratamiento medico (posicion levantada 45 grados C, dieta espesa y fraccionada y metoclopramida 0.3 mg/kg/dia) mejoraron clinicamente 81 enfermos (64,2% de ellos en menos de 6 meses). 9 fueron intervenidos quirurgicamente. El tratamiento medico fue seguido estrictamente irregularmente o rechazado en 62.2%; 26.7% y 11.1% respectivamente. El esofagograma de control 8-12 meses despues de iniciado el tratamiento fue normal en 40 ninos y demostro RGE en 24 ninos. De los 81 recuperados clinicamente la relacion peso/talla al ingreso y al termino de la experiencia fue bajo el 25 percentil en 32% y 18.6% respectivamente. 26 ninos abandonaron los controles, siendo asintomaticos

Isoprinosine en la hepatitis viral aguda tipo A. Comunicacion preliminar. / Isoprinosine in acute viral hepatitis type A.Preliminary report

Ante el aumento progresivo de la hepatitis viral aguda Tipo A y la necesidad de aminorar la magnitud del impacto medico social, economico, laboral, de ausentismo escolar y factores epidemiologicos de esta enfermedad.Se estudio el efecto del isoprinosine sobre la evolucion clinica, histologica y de laboratorio en 13 ninos sin demostrar ventajas con respecto al uso de placebo