[Biliary atresia and polysplenia syndrome: outcome and growth post-transplantation]. / Atresia de vías biliares y síndrome de poliesplenia: evolución y crecimiento postrasplante.

Biliary atresia (BA) is the most common indication for liver transplantation in children. Approximately 7-10% of these patients have the associated polysplenia syndrome (PS). The prognosis of patients with BA and PS has been reported to be poorer than that in patients with BA without PS. All patients who underwent liver transplantation for BA and who still attend periodic controls at the outpatient clinic were considered. A retrospective study of outcome and growth in children with BA was made, and compared with a subgroup of patients with BA and PS. There were no significant differences on complications, liver and renal function tests, lipids and growth data. We concluded that BA and PS do not preclude successful liver transplantation.

Hematologic abnormalities in liver-transplanted children during medium- to long-term follow-up.

OBJECTIVE: Hematologic abnormalities as adverse effects related to immunosuppressive drugs in liver-transplanted children are rarely reported. We have observed anemia, neutropenia, and thrombocytopenia in our pediatric liver-transplant population. The aim of this study was to exclude all suspected etiologies to define the association of immunosuppressants with these abnormalities. METHODS: Patients under 18 years old who still attend periodic controls at liver-transplant outpatient clinics were considered. Seventy patients met the inclusion criteria, 36 girls and 34 boys. Mean patient age was 5.6 years (range: 7 months to 17 years) and mean follow-up 6 years (range: 1-10 years). Medical records were reviewed beginning 1 month posttransplant. Treatment exposures, irradiation, blood product administration, and all laboratory studies were reviewed. When a hematologic abnormality was detected, we recorded the management for its resolution, the clinical response to therapy and the length of treatment. RESULTS: Twenty-five of the 70 children suffered 26 abnormal hematologic episodes (anemia 14, neutropenia 2, thrombocytopenia 3, simultaneous anemia and neutropenia 5, and pancytopenia 2). Eleven episodes (42%) had unclear etiologies and the process of elimination suggested an association with the immunosuppressant. Switching immunosuppressant was required in four patients and dose reduction in seven. CONCLUSIONS: Hematologic abnormalities in liver-transplanted children are common. The etiology is readily attributable to several causes. When the immunosuppressant appears to be a possible cause, the first step is dose reduction. If the hematologic abnormality persists despite dose reduction, a trial switch may be required.

[First orthotopic liver transplantation in patient with biliary atresia and situs inversus in spain]. / Primer trasplante hepático en un paciente con atresia de vías biliares y situs inversus en España.

Biliary atresia is the most common indication for liver transplantation in the pediatric age group. The Kasai portoenterostomy has become established as the primary treatment for biliary atresia. If portoenterostomy fails, death before 2 years of age is likely without liver transplantation. The most common multiple malformation syndrome associated with biliary atresia is polysplenia syndrome, which forms a constellation of defects of body symmetry, splenic development and vascular anomalies, including situs inversus, polysplenia and others. The situs inversus was formerly considered an absolute contraindication for liver transplantation. Recently however, several case reports have been published suggesting that neither situs inversus nor this particular subset of vascular abnormalities should be considered contraindications to liver transplantation. We present one case of liver transplantation performed in patient with biliary atresia, situs inversus and polysplenia. This is the first report described in Spain for a liver transplant in a child with biliary atresia plus situs inversus.

[Vaccinations and solid-organ transplantation: review and recommendations]. / Vacunaciones y trasplante de órgano sólido: revisión y recomendaciones.

Pediatric solid-organ transplant recipients are at high risk for various infectious diseases. Many children are not fully vaccinated before transplantation. To reduce the risk of morbidity and mortality from vaccine-preventable disease, physicians treating pediatric solid-organ transplant recipients should monitor the immunization status of these patients. Consensus on the most appropriate immunization schedule for solid-organ transplant recipients is lacking. Therefore, we provide a review of the currently available data on immunization safety and efficacy and describe strategies to avoid vaccine-preventable diseases in pediatric solid-organ transplant recipients.

Vacunaciones y trasplante de órgano sólido: revisión y recomendaciones / Vaccinations and solid-organ transplantation: review and recommendations

Los receptores pediátricos de trasplante de órgano sólido tienen un alto riesgo de padecer diversas enfermedades infecciosas. Algunos niños no han recibido las vacunas recomendadas para su edad, antes del trasplante. Para reducir el riesgo de morbilidad y mortalidad de las enfermedades prevenibles mediante vacunación, es importante que los médicos que tratan a estos niños vigilen su estado vacunal. No hay consenso sobre el esquema de inmunización más apropiado para los receptores de trasplante de órgano sólido, por lo que se revisa la información disponible sobre la seguridad y la eficacia de las inmunizaciones, y se describen las estrategias para evitar las enfermedades prevenibles mediante vacunación en los niños receptores de trasplante de órgano sólido. (AU)

[Acute liver failure due to T cell lymphoma without hepatic infiltration]. / Insuficiencia hepática aguda por linfoma de células T sin infiltración hepática.

Hepatomegaly and alterations in hepatic function are common to all patients with sickle-cell disease. In these patients, hepatic sickling is a manifestation of severe intrahepatic vaso-oclusive crises, even at levels of 25 % HbS and hematocrits of more than 45-50 %, which in 10 % of cases can lead to acute hepatic failure (AHF). AHF can be due to a variety of causes, including hematologic malignancies, but T cell lymphoma, which is usually secondary to diffuse hepatic infiltration and ischemia, is an exceptional cause, although other mechanisms can be involved. Cytokines released by lymphomas have recently been implicated as a cause of AHF.We describe a black girl with sickle cell disease, who developed AHF due to T cell lymphoma without lymphomatous infiltration of the liver. The only mechanism found to explain the clinical findings was release of cytokines by lymphoma. In patients with AHF of unknown etiology we propose early liver biopsy, because prognosis depends on the presence or absence of hepatic tumour infiltration. If AHF develops in a patient with diagnosed malignant disease, cytokine release may be the cause of AHF. Consequently, early diagnosis of the underlying disease and provision of liver support, as well as direct removal of inflammatory mediators from the circulation by exchange transfusion or other methods, should be the main priorities.

Primer trasplante hepático en un paciente con atresia de vías biliares y situs inversus en España / First orthotopic liver transplantation in patient with biliary atresia and situs inversus in Spain

La atresia de vías biliares es la indicación más frecuente para trasplante hepático en la edad pediátrica. La portoenterostomía de Kasai es el tratamiento primario para la atresia de vías biliares. Si la portoenterostomía falla, el fallecimiento suele ocurrir antes de los 2años de edad sin el trasplante hepático. El síndrome de múltiples malformaciones más comunmente asociado con la atresia de vías biliares es el síndrome de poliesplenia, formado por una constelación de defectos en la simetría corporal, desarrollo del bazo y anomalías vasculares, incluyendo situs inversus, poliesplenia y otros. El situs inversus era considerado como una contraindicación absoluta para el trasplante hepático. Sin embargo recientemente han sido publicados varios casos, sugiriendo que ni el situs inversus ni la presencia de dichas alteraciones vasculares deben ser consideradas como contraindicaciones para el trasplante hepático. Presentamos un caso de trasplante hepático realizado a un paciente con atresia de vías biliares, situs inversus y poliesplenia. Es el primer caso descrito en España (AU)

Insuficiencia hepática aguda por linfoma de células T sin infiltración hepática / Acute liver failure due to T cell lymphoma without hepatic infiltration

En pacientes con anemia de células falciformes, la hepatomegalia y alteración de la función hepática son comunes. En ellos, las crisis vasooclusivas intrahepáticas graves, pueden ocurrir con niveles de hemoglobina del 25 per cent y hematocrito superior a 45-50 per cent. Un 10 per cent pueden llegar a fallo hepático agudo (FHA). El FHA tiene diversas causas, entre ellas los procesos hematológicos malignos, excepcionalmente el linfoma de células T, que por lo general produce infiltración hepática e isquemia, aunque éste no es el único mecanismo lesional. Recientemente se ha considerado a las citocinas liberadas por linfomas como causa de FHA. Se presenta el caso de una niña de raza negra, con enfermedad de células falciformes, que presentó FHA secundario a un linfoma de células T que no infiltró al hígado, y donde el único mecanismo que se encontró para explicar el cuadro clínico fue el mediado por las citocinas. Cuando los estudios de imagen no confirman la infiltración hepática por células malignas, debe valorarse realizar una biopsia hepática, pues el pronóstico del FHA variará en función de si hay o no infiltración tumoral. Si la causa del FHA es compatible con una enfermedad neoplásica, las citocinas pueden ser las responsables del FHA. Por ello, establecer medidas de soporte de la función hepática e iniciar tratamiento precoz de la enfermedad de base, además de remover directamente estos mediadores inflamatorios de la circulación mediante exanguinotransfusión u otro procedimiento debe constituir la principal preocupación (AU)

[Iron supplementation in a child with severe burns]. / Suplementación férrica en quemaduras graves.

Non-intentional burns are still a frequent cause of morbidity in children despite the implementation of preventive campaigns. Children with severe burns (more than 10 % of the body surface) may require iron supplementation, especially in the post-stress or recovery phase. We report the case of a 15-month-old boy, who suffered polytrauma and burns to 20 % of the body surface. A dermal graft was performed eight days after his admission to the pediatric burns unit, which required transfusion of packed red blood cells. On day 15 hemoglobin value was 9.3 g/dL, the reticulocyte hemoglobin content was reduced to 26.2 pg (normal value: 27.2-30.3 pg) and the reticulocyte mean corpuscular value was increased to 124 fl (normal value: 101-108 fl). These findings were interpreted as a functional iron insufficiency, despite the increased ferritin value (219 ng/mL). We highlight the satisfactory outcome of the different hematologic values, with the development of reticulocyte crisis and normalization of hematological parameters after administration of 0.5 mg/kg/day of ferrous sulfate, plus iron corresponding to the follow-up milk formula and a regular diet for the child's age.

Suplementación férrica en quemaduras graves / Iron supplementation in a child with severe burns

Las quemaduras de causa accidental continúan afectando de forma frecuente a los niños, a pesar de las diversas campañas de prevención que se llevan a cabo. El niño afectado de quemaduras graves (más del 10% de superficie corporal quemada) puede requerir una suplementación férrica adecuada, sobre todo en la fase postestrés o de recuperación. Se describe el caso de un niño varón de 15 meses de edad afectado de politraumatismo y quemaduras del 20% de extensión corporal. Al octavo día de su ingreso en la Unidad de Quemados pediátrica se practicó autoinjerto laminar, precisando transfusión de concentrado de hematíes. A los 15 días de evolución mostraba una hemoglobina disminuida de 9,3 g/dl, contenido de hemoglobina reticulocitaria disminuida de 26,2 pg (valores normales [VN], 27,2-30 pg) y volumen corpuscular medio reticulocitario elevado de 124 fl (VN, 101-108 fl), por lo que a pesar de unos valores elevados de ferritina (219 mg/ml) se interpretó como insuficiencia funcional del hierro. Se destaca la evolución satisfactoria hematológica, con aparición de crisis reticulocitaria y normalización de los parámetros hematológicos, con la administración únicamente de 0,5 mg/kg/día de sulfato ferroso más el hierro correspondiente a la fórmula de continuación y la alimentación normal para su edad (AU)

[Branhamella catarrhalis: its respiratory pathogenicity in childhood]. / Branhamella catarrhalis: estudio de su patogenicidad respiratoria en la edad pediátrica.

Branhamella catarrhalis is a nasopharyngeal commensal which is being increasingly recognised as a pathogen, causing mainly infective exacerbations of chronic lung disease. It can also originate serious infections, like septicaemia, in patients with chronic predisposing conditions. During the period from 1979 to 1987, 22,501 respiratory tract samples from children were evaluated. Ninety nine isolated of Branhamella catarrhalis were identified (0.44%). Patients' age extended from 12 days to 9 years, with patients younger than two years representing 73%. Sixty three out of 77 cases investigated (82%) were positive for beta-lactamase. The most frequent finding was the recovery of Branhamella catarrhalis in tracheal aspirates from children with a tracheotomy or prolonged nasotracheal intubation. One of these children had a septic episode during which Branhamella catarrhalis was isolated from blood. Also remarkable is a case of pneumonia in a patient with congenital hypogammaglobulinaemia. Branhamella catarrhalis was also recovered in a wide variety of acute upper and lower respiratory tract infections in children without previous predisposing conditions. It is less clear its pathogenic role in these cases.

[Cardiac tamponade in children as a complication of central venous catheterization. Report of four cases]. / Taponamiento cardiaco en niños como complicación de la cateterización venosa céntral. Presentación de cuatro casos.

Very few cases of children with cardiac tamponade from central venous catheterization have been reported. Four cases are described. In 3 patients the diagnosis was suspected, a pericardiocentesis was performed and they survived. The other patient died and, at autopsy, diagnosis was made. The available literature on cardiac tamponade from central venous catheters in children is reviewed, preventive measures are considered in detail and the need for a high index of suspicion is highlighted.

Effect of verapamil in infants with paroxysmal supraventricular tachycardia.

Twenty-nine consecutive spontaneous attacks of paroxysmal supraventricular tachycardia (PSVT) in 14 infants (mean age 4.4 months) were treated with verapamil. No infant had associated heart disease. Verapamil 1-2 mg i.v was administered over 30 seconds. The dosage varied according to the weight of the infant. Within 60 seconds sinus rhythm was obtained in 28 instances (96.5%). No significant complications were observed. The high effectiveness, rapid action and lack of undesirable side effects observed in this series suggest that verapamil is the drug of choice in the treatment of PSVT in infants without underlying heart disease.