Maravillas Díaz

Long-term survival expectancy after liver transplantation in children

PURPOSE The aim of this study was to assess the long-term survival rate in children who have undergone orthotopic liver transplantation (OLT) in the last 13 years. METHODS The records of 198 consecutive patients under 18 years of age who underwent 249 OLTs between 1986 and 1998 were reviewed. Actuarial patient survival rates were assessed at 1, 3, 5, and 10 years in the whole series, in the last 5 years, and in patients surviving more than 1 year. Age, weight, and indications were analyzed, as well as type and incidence of posttransplant complications. The median follow-up period was 41 months (0 to 154 months). RESULTS Biliary atresia was the most common indication (41.9%) followed by alpha-1 antitrypsin deficiency (8.1%), Alagille syndrome (7.6%), and fulminant hepatic failure (6.6%). One hundred forty-six patients (58.6%) were below 5 years, and 46 patients were (18.5%) younger than 1 year at operation. Sixty-eight patients (27.3%) weighed less than 10 kg. One hundred seventy whole organs and 70 reduced, 5 living-related donor, and 4 split-liver allografts were used. Hepatic artery thrombosis (n = 18), primary nonfunction (n = 15), and chronic rejection (n = 14) were the most common causes for allograft failure. Fourteen patients (7%) had posttransplant lymphoproliferative disorders (PTLD) at a median time of 28 months (4 to 124 months) postoperation (3 died). The 1-, 3-, 5-, and 10-year actuarial patient survival rates are 80%, 76%, 74%, and 74%, respectively; over the last 5 years it is 88% at 1 year and 82% at 3 and 5 years. For patients surviving more than 1 year, 3-, 5-, and 10-year actuarial survival rates are 95%, 93%, and 93%, respectively. CONCLUSIONS (1) Overall results of OLT improve with increasing experience. (2) Children who survive more than 1 year after OLT have an excellent prognosis, although long-term complications of immunosuppression can be expected.

Late biliary complications in pediatric liver transplantation

PURPOSE The aim of this study was to review the biliary complications occurring in late follow-up after liver transplantation in children. METHODS The medical records of 135 children who received orthotopic liver transplantations (OLT) and had graft survival of more than 1 year were reviewed. Technical variants using a reduced-size graft were applied in 32 (23.7%). For biliary reconstruction, 15 patients had choledochocholedochostomy and 120 a Roux-en-Y loop. Biliary reoperation in the early post-OLT period was needed in 24 patients (17.7%). Routine checking of liver function and duplex Doppler ultrasonography (DDS) were performed during the follow-up period, which averaged 58 months. Late biliary complication was defined as that occurring after the first hospital discharge. RESULTS Late biliary complications occurred in 18 children (13.3%); 16 showed symptoms or analytical disturbances in liver function tests. The Diagnoses included uncomplicated cholangitis (n = 6), anastomotic biliary stricture (n = 7), ischaemic damage of the biliary tree (n = 3) including one late (28 months) hepatic artery thrombosis leading to an intrahepatic biloma. and bile leak after T-tube removal (n = 2). The six children with uncomplicated cholangitis had no repeat episodes in follow-up despite persistent aerobilia. Six patients affected by anastomotic strictures were treated successfully with percutaneous dilatation and, if present, stone removal. Persisting dysfunction and cholangitis occurred in one case affected by ischaemic biliary disease. Biliary leaks after T tube removal settled spontaneously. Risk factors for late biliary complications were determined. There was no relation to the cold ischaemia time, type of graft or biliary reconstruction, or previous early post-OLT biliary reoperation. Aerobilia (affecting 21.5% of OLT patients) was related to cholangitis (P = .001). CONCLUSIONS Anastomotic strictures, reflux of intestinal contents via the Roux-en-Y loop, and residual ischaemic damage led to late biliary complications in 12% of paediatric OLT patients. Evidence of biliary dilatation on DDS may be delayed in anastomotic strictures; in these cases the results of percutaneous treatment were excellent. Children with aerobilia have and increased risk of cholangitis.

Donor vascular grafts for arterial reconstruction in pediatric liver transplantation

The authors compared the results of 48 orthotopic liver transplantations (OLT) in which revascularization was achieved with a conduit interposed between the receptor aorta and the graft (vascular graft [VG] group) with those obtained for 56 OLT performed during the same period (1991 to 1994) in which end-to-end anastomosis (EEA) of the hepatic arteries or celiac trunk was used (EEA group). In the VG group, the interposed conduits were the cadaveric iliac artery (37) the living-donor saphenous vein (3), or nonthrombosed conduits from previous transplants (8) (7 iliac arteries, 1 saphenous vein). There were significant differences between the two groups with respect to recipient age, recipient weight, the retransplant:first transplant ratio, the number of emergency transplantations, the use of reduced-size grafts, and intraoperative transfusion requirements. Twenty-nine grafts in the VG group (60.4%) and 43 in the EEA group (76.7%) currently are functioning. The actuarial 3-year graft survival rates are 60% and 71.5% for the VG and EEA groups (P < .05), respectively. The rate of arterial thrombosis did not differ between the two groups. The authors conclude that, although EEA of the hepatic artery is still the preferred revascularization technique for OLT, revascularization of the liver graft by conduit interposition is safe when EEA is not possible. Reutilization of the interposed conduit during retransplantation proved to be safe in the absence of hepatic artery thrombosis.

Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment

Abstract The outcome of 18 biliary atresia (BA) patients (5 male, 13 female; age range 10.7–22.5 years; mean 15.4±0.7 years) treated with hepatic portoenterostomy (HPE) and jaundice-free for more than 10 years without liver transplantation (LT) is analyzed retrospectively. Eight of these patients subsequently required LT (age at LT 12.8±0.5 years, range 10.5–15.2 years); 3 children (aged 11.6, 13.2 and 14.1 years, respectively) had episodes of gastrointestinal variceal bleeding associated with other signs of severe disease and are now candidates for LT; and among the 7 asymptomatic patients (age range 11.2–22.5 years; mean 15.9±2.1 years), 5 had sonographic and biochemical signs of moderate portal hypertension (PH). In order to analyze whether the age at transplantation influences the survival of children transplanted for BA, we also reviewed the outcome of 71 BA patients transplanted at our hospital between 1986 and 1996. All the children older than 10 years at the time of LT were alive; only patients younger than 10 years died following LT (n= 15). We conclude that the natural outcome of extrahepatic BA is toward PH, fibrosis, and cirrhosis, even in those cases successfully treated with HPE. In our experience, the results of sequential treatment with HPE and LT were excellent.

Cyclosporine monitoring in the early post-transplant period in pediatric liver transplant recipients

Abstract:  Monitoring of CsA blood levels two h post‐dose (C2) has shown a higher correlation to drug exposure than monitoring of trough levels (C0) at least in adults, but initial doses and target blood levels of CsA have yet to be established in pediatric transplant patients. The objectives of the study were to describe the pharmacokinetics of CsA administered by NGT in the first days after transplantation and the dose of Sandimmun Neoral® required to achieve minimum therapeutic range blood levels. This study included 20 pediatric liver transplant recipients (mean age of 3.2 yr) treated with CsA administered by NGT from day one post‐transplant until they were able to ingest oral medication. The study was continued until one yr of post‐transplant follow‐up. Eight h pharmacokinetic profiles were performed on days one, three, and five post‐transplant to determine the minimum dose required to achieve the therapeutic range. All children received an initial dose of 15 mg/kg/day of CsA by NGT. Mean CsA doses administered on days one, three, and five were 16.8, 29.5, and 36.5 mg/kg/day, respectively. Mean C0 levels of 119, 310, and 337 ng/mL and mean C2 levels of 213, 753, and 888 ng/mL were obtained. No correlation was found between C0 and C2 levels and the AUC0–8 h. Intravenous administration of CsA was required in 55% of patients. The biopsy‐confirmed acute rejection rate was 45%, with graft and patient survival rates of 95 and 100%, respectively. Conclusions: Poor absorption of CsA in small children requires a considerable increase in dose. CsA exposure cannot be estimated by single C0 or C2 determinations in the early post‐transplant period.

Pediatric living donor liver transplantation

AIM The aim of this study was to analyze the results of living donor in a pediatric liver transplantation program. PATIENTS Twenty-six living donor liver transplantations were performed in children from 0.5 to 14.8 years of age. The main indication was biliary atresia (72%) followed by tumors (2 hepatoblastomas and 1 hepatocarcinoma). Left lateral segments were used in 23 (1 transformed into a monosegment), 1 left lobe was used in 1, and right lobes were used in 2. Arterial reconstruction employed saphenous venous grafts in the first 3 cases and end-to-end anastomoses with a microsurgical technique in the following 22 cases. RESULTS There has been no major morbidity in the donors, with a median hospitalization of 6 days. Four grafts have been lost; 2 in the first 3 cases. In only 1 case, the graft loss was related to the procedure saphenous venous graft thrombosis). Early biliary complications were frequent (23%). Six month, 1 year, and 5 year graft and patient survival rates were 91%, 85%, and 85% and 100%, 96%, and 96%, respectively. CONCLUSIONS Living donor liver transplantation is an excellent option for transplantation in children.

Tacrolimus for steroiD‐resistant liver rejection in children

Abstract Eighteen pediatric liver transplant recipients were converted from cyclosporine‐based immunosuppression to tacrolimus for refractory rejection episodes affecting 21 grafts. Before conversion, steroid boluses were applied to all episodes followed by OKT3 monoclonal antibodies in 3 of them. Baseline biopsy showed cellular rejection in 18 patients and ductopenia in 3 cases. Thirteen episodes initiated within the first 2 postoperative weeks, and 8 occurred beyond the 21st day. A previous steroiD‐responsive episode of rejection was noted in 4 patients. Tacrolimus was administered by the oral route to obtain trough blood levels in the range 6–15 ng/ml. Reversal of rejection was obtained in 15 patients (71.4%). Complete normalization of liver function tests was achieved in 10 out of 12 patients who were followed for more than 6 months. A refractory evolution affected 6 patients (28.5 %). Significant factors predictive for tacrolimus‐resistant rejection were identified as ductopenia on baseline biopsy, previous episodes of acute rejection, late onset rejection (beyond 21st posttransplant (day), and a longer time of evolution of rejection prior to conversion.

Growth and height in children after liver transplantation.

Abstract  To assess the linear growth after liver transplantation, height curves were constructed for 45 children who underwent liver transplantation at the Childrens Hospital 19 La Pa 9, Madrid, and were followed for more than 2 years. The prednisolone dose was progressively tapered and switched to alternate‐day administration at 12 months. Growth was severely impaired during daily steroid therapy but the mean growth rate normalized in the second year and a significant improvement was observed in successive years. Observations over a long period revealed flucting growth rates under stable or decreasing doses of prednisolone on alternate‐day administration. Beyond the first year, some annual periods of abnormal growth rate occurred in 57 % of the children. Marginally better posttransplantation growth was observed in children transplanted for intrahepatic chole‐static diseases. The prednisolone dose did not correlate with growth rate. In the long term, short stature was highly prevalent due to an accumulation of factors: previous disease, daily prednisolone period, inconstant growth rate under alternate‐day steroid therapy, and puber‐tal delay.

La educación musical en la etapa 0-6 años

Para potenciar y desarrollar las capacidades de expresion y las necesidades de comunicacion que todos los ninos y ninas muestran desde el momento de su nacimiento, padres, madres y educadores desempenan un importante papel. Prepararse especialmente para poder realizar una labor de responsabilidad como es la de ser guia y educador de su sensibilidad musical, es una gran tarea.

A new Spanish-language questionnaire for musical self-concept

The aim of this study is to analyze the psychometric properties of the Musical Self-Concept Questionnaire (CAMU), an abbreviated and culturally adapted Spanish language version of the Music Self-Perception Inventory (MUSPI) developed by Vispoel. Participants comprised 1,126 students from professional and advanced conservatories located in different autonomous regions of Spain. The internal consistency of the instrument was found to be high (Cronbach’s alpha = .907; AVE = .685). The exploratory factor analysis revealed an internal structure consisting of seven factors: singing, playing, reading, composing, listening, moving and general musical self-concept. Sex differences and correlations between musical self-concept and both verbal self-concept and causal attributions confirm the criterion validity of the questionnaire. In addition to being a useful tool for measuring musical self-concept in Spanish, the CAMU also enables cross-cultural research into this construct to be conducted, in line with that carried out in English.