Gloria Ríos M

Rescate con Sirolimus por rechazo agudo recurrente y diabetes mellitus en trasplante hepático: Caso clínico pediátrico

Sirolimus (SRL) is a new immunosuppressive drug approved for renal transplantation, but is being used increasingly in orthotopic liver transplantation (OLT). Compared with the calcineurin inhibitors, SRL has different mechanisms of action and side effects profile. Thus, this drug offers significant potential advantages over other immunosuppressive agents. SRL does not cause glucose intolerance, hypertension or renal failure, but it may cause dyslipidemia, hepatic artery thrombosis, thrombocytopenia, anemia, leukopenia, oral mucosa ulcers, edema, arthralgias and wound complications. SRL inhibits the signal of interleukin 2 at a post-receptor level, inhibiting lymphocyte proliferation and fibroblast proliferation. It also has antineoplastic and antifungal effects. We report a 10 years old girl who underwent OLT, experiencing a biopsy-proven recurrent acute rejection (AR) in spite of using three immunosuppressive agents (tacrolimus, mofetil micofenolate and steroids). She developed diabetes mellitus as a consequence of the immunosuppressive therapy. She was rescued with SRL, not experiencing AR again. Mofetil micofenolate, steroids and insulin could be discontinued and tacrolimus doses were reduced, without experiencing severe complications. SRL is a new and safe immunosuppressive agent for rescue in patients with OLT and recurrent AR.

Enfermedad celíaca en el adolescente y adulto joven.: Un desafío para gastroenterólogos de niños y adultos

Background: Follow-up of celiac patients in Chile is often interrupted when adolescents are referred to adult gastroenterologists. Aim: To study the evolution of patients with celiac disease when they reach adolescence or young adulthood. Patients and methods: Current adherence to gluten-free diet and its relation to symptoms and circulating antiendomysial antibodies were evaluated in the 58 confirmed celiac patients older than 12 years of age controlled at 3 hospitals in Santiago. Results: Mean age at the moment of this assessment was 17.8 ± 5 years, 65.5% were women, 12.5% were at nutritional risk (-IDS) while 20% were overweight/obese. Although all patients declared themselves asymptomatic, a focused questionnaire revealed that 26% suffered some symptoms. Only 24.1% followed a strict gluten-free diet. Eight of 20 patients who ate gluten-containing diets had negative antiendomysial antibodies (EMA), three of whom turned positive within 6 to 9 months. In three of four (asymptomatic) cases that accepted a new jejunal biopsy, histology was abnormal. One patient who followed a strict diet had EMA (+) and normal histology. Conclusions: These results confirm that after childhood, symptoms abate significantly in celiac patients. The observed sensitivity and specificity of EMA makes necessary to maintain small intestinal biopsies as the gold standard for diagnosis and confirmation of the disease. (Rev Med Chile 2001; 743-8)

Tiempo de exposición al gluten y marcadores de riesgo de diabetes mellitus insulino dependiente en pacientes celíacos

158 celiac patients were classified as: G1, (n=30 patients)studied at the time of diagnosis; G2 (n=97 patients) exposed to gluten as a result of non compliancewith the gluten free diet and, G3 (n=31 patients) who had maintained a long term, strict gluten freediet. Isotype IgG anti-islet cell antibodies were detected by indirect immunofluorescence using mon-key pancreas; results were reported in Juvenile Diabetes Foundation (JDF) units.

Anticuerpos anti-transglutaminasa tisular: ¿una mejor prueba para detectar transgresiones menores de la dieta en pacientes celíacos?

Background: Endomysium antibodies (EMA) do not detect minor dietary transgressions in patients with celiac disease. Aim: To compare the sensitivity and specificity of tissue transglutaminase antibodies (tTGA) and endomysium antibodies (EMA) in biopsy proven celiac patients at the time of diagnosis and during gluten free diet (GFD). Patients and methods: One hundred fifty three subjects were studied: a) 30 healthy controls; b) 9 cases with cows milk allergy; c) 24 celiac patients at time of diagnosis; d) 25 celiac patients adhering to the GFD; e) 65 celiac patients with poor/no adhesion to GFD. EMA and tTGA IgA were measured by immunofluorescence and ELISA, respectively. Results: Sensitivity and specificity were 100% and 97.4% for tTGA, respectively. All patients with cows milk allergy were EMA (-) and 8 of 9 (88.9%) were tTGA (-). In celiac patients not adhering to the GFD, EMA and tTGA positivity were similar (80% and 81,5%, respectively); 95,4% of the subjects tested positive for at least one of them. All patients adhering to GFD were EMA (-) but tTGA were (+) in 28% of them. Conclusions: EMA and tTGA have similar sensitivity and specificity at the time of diagnosis of celiac disease. Positive tTGA in 28% of patients that adhered strictly to the GFD and whose EMA were negative suggest that tTGA may be helpful in detecting minor dietary transgressions and should be further evaluated (Rev Med Chile 2003; 131: 25-29)

Falla hepática fulminante por parvovirus B 19 y trasplante hepático, caso clínico

Introduccion: La infeccion por parvovirus humano B19 (PHB 19) produce un amplio rango de enfermedades que van desde eritema infeccioso en ninos hasta artritis aguda en adultos. Algunos estudios sugieren un rol patogenico del PHB 19 en el desarrollo de la hepatitis aguda (HA) y falla hepatica fulminante (FHF) en ninos y adultos. La Anemia aplastica (AA) es una complicacion reconocida de la HA y FHF por PHB 19. Objetivo: Reportar un caso de FHF por infeccion por PHB 19 y revisar la literatura. Caso clinico: Nina de 7 anos de edad con HA que en una semana desarrollo FHF con serologia IgM anti-PHB 19 positiva. Otras causas virales, autoinmunes, metabolicas o toxicas fueron descartadas. Fue sometida a trasplante hepatico ortotopico (THO) y un ano despues no ha presentado complicaciones. Conclusiones: El PHB 19 puede causar HA y FHF, su oportuno diagnostico y tratamiento, que en el caso de la FHF incluye el THO puede resultar en un pronostico favorable

Hiperplasia nodular focal del hígado en niños y Trasplante Hepático

La Hiperplasia Nodular Focal (HNF) del higado es una tumoracion benigna probablemente secundaria a una alteracion en la vascularizacion del parenquima hepatico, rara en la edad pediatrica. Objetivo: Reportar un caso clinico de HNF que requirio trasplante hepatico ortotopico (THO) y revisar la literatura actual con respecto a la conducta terapeutica a seguir. Caso clinico: Nino de 15 anos de edad con una masa abdominal palpable. Ecografia y tomografia axial computada de abdomen demostraron un tumor multifocal en ambos lobulos hepaticos de caracteristicas irresecables. Biopsia operatoria del higado confirmo una HNF. El paciente desarrollo hipertension portal debido a compresion tumoral de la vena porta, con ascitis, encefalopatia hepatica e insuficiencia renal, por lo que se decidio su manejo mediante un THO en injerto total, con una excelente evolucion a 5 anos de seguimiento. Conclusion: La decision entre un enfoque conservador o quirurgico depende de las caracteristicas de cada caso, siendo el THO una alternativa terapeutica en pacientes con tumores hepaticos benignos e irresecables

Trasplante combinado hepato-renal: a propósito de un caso

End stage renal disease is not an absolute contraindication for liv-er transplantation (LT) in patients with end stage liver disease. Actuarial patient and graft sur-vival are comparable for children and adults who undergo LT alone and liver-kidney trans-plantation (LKT). The most common indications for LKT are the primary hyperoxaluria type I(PH1) and the liver and renal polycystic disease. We report a 12 years old boy with congenitalhepatic fibrosis with severe portal hypertension, encephalopathy and polycystic kidney diseasewith end stage renal disease on dialysis that underwent LKT. During the second postoperativeweek, he had a biopsy-proven acute liver and renal rejection, that had a good response to corti-costeroids. Thirty days after surgery, the liver biopsy was without rejection. No other complica-tions were observed (Rev Med Chile 2003; 131: 1309-12).(