117,659 research outputs found
Liver and cardiac function in the long term after Fontan operation.
No full textAnn Thorac Surg. 2008 Jul;86(1):177-82.
Liver and cardiac function in the long term after Fontan operation.
Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D'Antiga L.
Source
Department of Pediatric Hepatology, University of Padova, Padova, Italy.
Abstract
BACKGROUND:
Patients who underwent Fontan operation have some degree of liver disease. We aimed to assess the long-term liver and cardiac function after Fontan operation.
METHODS:
Patients enrolled underwent physical examination, biochemical tests (aspartate aminotransferase, alanine aminotransferase, gamma glutamyl transpeptidase, bilirubin, international normalized ratio, coagulation factor V, protein profile, fecal alpha-1-antitrypsin), echocardiogram, and liver ultrasonography. A liver disease score was adopted to compare the degree of liver involvement with hemodynamic features.
RESULTS:
The study enrolled 34 patients, median age 14.7 years (range, 4.1 to 26.7), 26 with a residual left ventricle, 8 with a residual right ventricle, affected by tricuspid atresia (17), pulmonary atresia (4), hypoplastic left heart syndrome (5), double-outlet right ventricle (2), single left ventricle (2), and miscellaneous (4), with median follow-up of 11.5 years (range, 1.7 to 23.3). We found hepatomegaly in 18 of 34 (53%), splenomegaly in 3 of 33 (9%), abnormal transaminases in 10 of 33 (30%), elevated gamma GT in 19 of 31 (61%), elevated bilirubin in 10 of 31 (32%), coagulopathy in 17 of 29 (58%), and protein-losing enteropathy in 4 of 21 (19%). Median heart rate z-score was -1.72. Hepatic dysfunction was strictly correlated to low cardiac index (r(2) = 0.34, p = 0.008) and to a lesser extent to reduced heart rate (r(2) = 0.18, p = 0.07).
CONCLUSIONS:
In children who underwent Fontan operation, hepatic dysfunction is correlated with low cardiac index and reduced heart rate. Maintaining or reestablishing a normal cardiac index might prevent or reduce liver disease in the long-term.
PMID:
18573420
[PubMed - indexed for MEDLINE
Atresia delle vie biliari extraepatiche: problema non solo chirurgico.
No full textDopo una revisione critica della letteratura sull'argomento, vengono riportati i risultati dell'intervento di epato-porto-enterostomia nella cura dell'atresia delle vie biliari extraepatiche, di tipo « non correggibile ». Sono anche elencate le complicanze, alcune legate direttamente all'intervento come quelle post operatorie precoci e l 'angiocolite, altre, come l'evoluzione cirrotica e l'ipertensione portale, più probabilmente connesse con la malattia, di base, inoltre, sulla base dell'esperienza personale circa i molteplici problemi chirurgici e medici,di questi, pazienti viene proposto oltre a uno schema di follow-up, uno schema di trattamento post operatorio di tipo medico. Esso include provvedimenti di tipo dietetico (apporto lipidico in parte sotto forma di MCT) e di tipo farmacologico (uso del fenobarbital come coleretico per l a profilassi dell'angiocolite e supplemento di vitamine liposolubili per Via parenterale). Per la sorveglianza della malattia è suggerito un accurato monitoraggio per la colangite , l' ipertensione portale e la evoluzione istologica dell'epatopatia
Progetti personalizzati per le famiglie multi-problematiche con minori: una sperimentazione promossa dalla Regione Abruzzo
No full textLong-term outcome of bone mineral density in children who underwent a successful liver transplantation
No full textAbstract
BACKGROUND:
It has previously been shown that bone mineral density (BMD) during the first year after orthotopic liver transplantation (OLT) in children with osteodystrophy increases remarkably and according to height. The effect of posttransplant factors possibly influencing bone mass in the long-term after a successful OLT in children is unknown.
METHODS:
Eighteen patients (9 male), median age 13.3 (range 4.7-23.7) years, median time after OLT 8.3 (1.1-17.3) years were enrolled. Indications for OLT were biliary atresia (8), Alagille (3), hepatoblastoma (2), NonA-NonG acute liver failure (2), intrahepatic cholestasis, cryptogenic cirrhosis, and cholesteryl-ester disease (1 each). At OLT, all were prepubertal and 12 were severely cholestatic. We recorded anthropometric data, immunosuppression, dual-energy x-ray absorptiometry (DXA), biochemical markers of bone metabolism, and liver function.
RESULTS:
Six children were on steroid therapy, eight were on cyclosporine, nine on tacrolimus. Median L1 to L4 spinal BMD was 0.720 (range 0.524-1.127) g/cm3, Z score -0.70 (-2.2- +2.1), height Z score -0.31 (-1.83- +1.96). Median bone mineral apparent density was 0.112 (0.084-0.142) (normal value 0.10-0.14) g/cm3. Median alanine aminotransferase level was 22 (range 11-79) IU/L, urinary free deoxypyridinolines 20.6 (7.1-62) nmol/mmol creatinine, osteocalcin 14 (2.3-45) microg/L, parathyroid hormone 51 (2-87) ng/L, Vitamin D3 67 (17-102) nmol/L.
CONCLUSION:
BMD after the first year from a successful pediatric liver transplantation is normal. Our study suggests that normal bone density in this setting is maintained for at least 1 decade.
Comment in
• Transplantation. 2005 Oct 27;80(8):1135-6.
• Transplantation. 2005 Oct 27;80(8):1135; author reply 1135-6
Iperfosfatasemia alcalina transitoria in bambini sottoposti a trapianto epatico. Esperienza di due casi.
No full text- …
