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J Larrauri

Publications and source records attributed to J Larrauri.

At least 19 recordsLinked to original sources
PubMed2005

[Nonalcoholic steatohepatitis, the enigma of bad progression].

Still, very little is known about the precise pathogenetic mechanisms, the triggering events and in particular, the evolution and treatment of nonalcoholic steatohepatitis (NASH). It is part of the broad spectrum of nonalcoholic fatty liver diseases (NAFLDs). Mainly, it has been reported as a benign disease, associated with metabolic disorders commonly occurrence en the general population. Nevertheless, the syndrome can lead to cirrhosis, liver failure or hepatocellular carcinoma, requiring liver transplantation. We present one patient with diagnosis of NASH, who was treated initially for overweight, HTA and hyperlipaemia with incompleted response and who showed a quickly progress to cirrhosis but no cause of liver decompensated disease could be identified. Currently she is at end-stage waiting a liver transplantation. Controlled and multicentric studies with the same definition of NASH and the study end-points are needed, and will provide information about diagnosis features and novel therapies to early management of the disease.

Adult↗
PubMed2004

[The multifocal hepatic hemangioendothelioma. Is always a benign tumor?].

UNLABELLED: The hepatic multicentric haemangioma is defined by its extension, affecting all the mass of the liver. The high mortality associated with it is mostly related with the complications produced by its enormous size (haemodynamic, platelet trapping, spontaneous rupture and bleeding). There is a general belief that is a benign tumor with possibility of spontaneous regression and cure. AIM: Retrospective analysis of our recent cases of MHH with the purpose of: 1 degrees) To show the evolution and results. 2 degrees) To realize if the "benign character" of the tumor is real or if some cases may be considered as malignant tumors. MATERIAL AND METHODS: 10 cases of MHH treated in the last 10 years. In 9 the age of presentation was less than 6 months and one patient was diagnosed at 3 and half years. The diagnosis was confirmed by image techniques in 7 cases and by biopsy in 3. In 7 patients extrahepatic vascular lesions were associated prior to the treatment. Methylprednisolone was given to all the cases and alpha-2-interferon was administered to the patients that not responded to the steroids. Vincristine was added to 2 patients. In two cases the hepatic artery embolization was tried and one patient had a liver transplant. RESULTS: Four children had at least one episode of congestive cardiac insufficiency, two patients suffered a consumption coagulopathy (Kasabach Merrit syndrome), and one presented acute hepatic failure. In six children it has been complete regression of the tumor, one more is still under treatment and three died. The dead were produced by the malignant behavior of the tumor in one case (tumoral rupture of a MHH recurrence in the transplanted liver), and possibly in other (intracranial haemorrhage and hepatic failure in a liver transplantation candidate without demonstrated extrahepatic extension in the previous studies, but with multiorgan dissemination at autopsy. In both cases it was impossible to discover signs of histologic or biologic malignancy neither in the primitive lesion nor in the metastasis. CONCLUSIONS: 1a) The regression of the MHH, spontaneous or induced by the treatment is frequent. 2a) Some cases of MHH are aggressive and develop local recurrences and distant metastasis. 3a) The discrimination between MHH of "benign" or "malignant" behaviour is not possible. 4a) Despite of the unpredictable biological conduct of the tumor, the liver transplantation must be considered as an option in the symptomatic cases that not respond to the conventional treatment.

Child, Preschool↗
PubMed2003

[Activity of a pediatric intestinal transplantation program in Spain].

AIM: To analyze the outcome of children with intestinal failure (IF) included as candidates for intestinal transplantation (IT). Patients, Eight out of 23 children with IF assessed since July 1997 met criteria for IT and were included on the waiting list. The causes of IT were Short Bowel Syndrome (SBS) in 6 and Microvillus Inclusion Disease (MID) in 2. The indication of IT were end stage liver disease (ESLD) in 5 (related to total parenteral nutrition administration, TPN), progressive hepatic fibrosis in 2 and loss of venous access in 1. The patients with ESLD were included for combined liver-small bowel transplantation (LSBT) and the remaining for isolated intestinal transplantation (IIT). RESULTS: Two children died waiting for LSBT, 4 patients are on the waiting list, 2 for LSBT and 2 for IIT (length of stay: 4-11 months). Two children were transplanted, one with IIT in a 2.5 years old boy with MID, and one with LSBT in a 22 months girl with SBS and ESLD. Both patients recovered intestinal function after transplantation and are a live (follow-up of 19 and 10 months respectively). The LSBT's patient is under treatment for postransplant lymphoproliferative disease (PTLD). CONCLUSIONS: The lack of suitable donors for the small children candidate to IT explains the long period of stay on the waiting list and the high pretransplant mortality. Two strategies are possible; early referral of children with IF to a transplant center and surgical techniques like ex vivi-hepatic reductions of the LSB graft.

Child↗
PubMed2001

[Intestinal transplantation. First experience in Spain].

UNLABELLED: We present the first case of intestinal transplant (IT) performed in Spacin. CASE REPORT: 28 months old boy with secretory diarrhea since the first month of life, diagnosed of microvillous inclusion disease (MID). He is on total parenteral nutrition (TPN) and had suffered of multiple episodes of catheter related sepsis with lost of standard venous access. An isolated small bowel transplant from a cadaveric donor was performed at the age of 3 years. The native ileocaecal valve and colon were not removed. RESULTS: Enteral feeding was started in the 2nd. week after the IT. On the 25th day he was off TPN. Since the 77th day, he eats regular foods by mouth. At the 6th month post IT the ileostomy was closed. Among the complications, he suffered a rotavirus infection on the 38th post IT day and an episode of mild rejection responsive to methil-prednisolone bolus. CONCLUSIONS: The IT is a therapeutic option that can be already offered with possibilities of success in our country. Although the colonic enterocytes express MVD, the recipient ileocaecal valve and colon can be preserved.

Child, Preschool↗
PubMed1999

A high index of suspicion: the key to an early diagnosis of Wilson's disease in childhood.

BACKGROUND: To study the clinical features of Wilson's disease in childhood. METHODS: Retrospective review of the clinical, laboratory, and histologic features and prognosis of Wilson's disease in 26 Spanish children. RESULTS: The first medical visit, at age 9.8+/-3.4 years (range, 4-16 years), was prompted by liver dysfunction detected accidentally (61%), symptoms of liver disease (27%), family screening (8%), and extrapyramidal symptoms and personality changes (4%). There were laboratory data of hepatic failure in 27%. All copper metabolism test results (total serum copper, 24-hour urine excretion, serum ceruloplasmin) were abnormal in 62%, two in 27%, and one in 11%. All patients in whom extrahepatic involvement was found at diagnosis had severe liver disease. Histologic findings were portal fibrosis with steatosis (29%), cirrhosis (21%), portal fibrosis (17%), chronic active hepatitis (17%), and minimal changes or normality (17%). Penicillamine was administered to all but one patient. Four children underwent liver transplantation, three of them having received penicillamine for 12, 45, and 70 days. Three other patients recovered from liver failure after 1 year of treatment with penicillamine. After a follow-up of 4.5+/-3.3 years, all the children survived. Penicillamine caused severe toxicity in one patient. CONCLUSIONS: Wilson's disease in childhood is generally detected by maintaining a high suspicion of liver disease in patients who have no or nonspecific hepatic symptoms. Kayser-Fleischer ring is rare in childhood. Drug therapy is effective and well tolerated, even in some cases of hepatic insufficiency.

Adolescent↗
PubMed1999

[Non-immunoallergic hepatotoxicity due to mesalazine].

Mesalazine is an aminosalicillic derivative considered as a safe alternative to the relative frequency (5-55%) of adverse effects observed with sulfasalazine. The well known hepatoxicity associated with sulfasalazine and attributed to its sulfamidic fraction is limited to few cases described in the treatment with mesalazine. We herein present a new case of hepatoxicity by mesalazine in a patient with lymphocytic colitis. The possible pathogenic mechanism is also commented upon.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1998

Chronic hepatitis C in children: a clinical and immunohistochemical comparative study with adult patients.

Limited information is available regarding the characteristics of the hepatitis C virus (HCV) infection in children. We compared the epidemiological background along with the virological and histological features as well as the intrahepatic immunologic phenotype of both children and adults with chronic hepatitis C (CHC). Serum samples of 24 pediatric and 32 adult patients were drawn for alanine transaminase (ALT) levels, HCV-typing, and viral load. The histological diagnosis and a semiquantitative immunohistochemical assessment were performed in all patients. The majority of children (62%) had been transfused and the mean duration of viral infection in these cases was 11 +/- 4 years, being similar in adults (11 +/- 9 years, not significant). Although genotype distribution was similar, viral load was lower in children than in adults. The mildest histological forms of chronic hepatitis along with a weak intrahepatic immunological phenotype were significantly more frequent among children than adult patients. In conclusion, in children with CHC, perinatal blood transfusion was the most frequent source of viral infection and the liver disease was characterized by both low ALT level and viral load, as well as the mildest histological and immunohistochemical forms of chronic hepatitis.

Adolescent↗
PubMed1998

Tacrolimus for steroid-resistant liver rejection in children.

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.

Adolescent↗
PubMed1998

Chronic hepatitis C in children: the pathological and clinical spectrum.

BACKGROUND & AIMS: Apart from the high-risk groups, the pathology of chronic hepatitis C in children is not well known. The aim of this study was to investigate the morphology of chronic hepatitis C in children without any underlying systemic disease and to evaluate its relationship to clinicovirological factors. METHODS: Liver biopsy specimens from 80 children positive for antibody to hepatitis C virus were evaluated using a semiquantitative scoring system. RESULTS: Chronic hepatitis was mild in most cases but had high-grade activity in 17 children (21.2%). A significant association was found between the grade of focal necrosis and alanine transaminase levels (P < 0.003). Fibrosis was absent in 22 cases (27.5%), mild in 44 (55%), and moderate in 13 (16.2%). Only 1 patient had cirrhosis. A significant relationship was detected between fibrosis scores and (1) duration of disease (P < 0.03); (2) portal inflammation (P < 0. 002); and (3) interface hepatitis (P < 0.003). CONCLUSIONS: In otherwise healthy children, chronic hepatitis C is a morphologically mild disease in most cases. Fibrosis increases with the duration of disease, suggesting that end-stage disease may develop in young adulthood. Alanine transaminase levels correlate with intralobular focal necrosis but not with other lesions. In this respect, liver biopsy retains its importance in the management of chronic hepatitis C in children.

Adolescent↗
PubMed1997

[Acute severe hypersensitivity reaction to salazopirin].

Sulphasalazine is the drug most widely used in intestinal inflammatory disease given its efficacy and low cost. Nonetheless, its administration is not lacking adverse effects. Two cases of severe hypersensitivity are herein described. Moreover, the diagnostic difficulties observed and their implication in the management of the underlying disease are discussed.

Adult↗
PubMed1995

Biliary atresia and the polysplenia syndrome: its impact on final outcome.

Up to 25% of babies with biliary atresia (BA) bear associated malformations that most often cluster in the polysplenia syndrome (PS). This article examines the impact of associated PS on the final outcome of the authors' BA patients and the necessary modifications of surgical technique during orthotopic liver transplantation (OLT) in these patients. The authors studied 88 consecutive infants with biliary atresia operated upon during the 10-year period between 1984 and 1993. Biliary drainage was provided by a Roux-en-Y hepaticojejunostomy. OLT was performed in 38. Eleven infants (12%) had three or more components of PS: malrotation (11), preduodenal portal vein (11), polysplenia (10), situs inversus (4), absent inferior vena cava (3), cardiac defects (2), and anomalous hepatic artery supply (2). Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versus 31%) are jaundice-free with a mean follow-up of 105 months. Three patients received transplants (2 from living-related donor) and are alive and well with normal liver function after a mean follow-up of 35 months. Overall survival was 72% in the BA plus PS and 66% in the BA group (P = not significant [NS]). Results show that long-term bile drainage in these PS patients can be achieved at least as often as in other BA patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attention to vascular anatomy is required to determine the feasibility of reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1995

Hepatic amyloidosis associated with systemic lupus erythematosus.

The association between amyloidosis and systemic lupus erythematosus has rarely been described. We report a case of a 37-year-old man with a long-standing SLE who developed clinical and laboratory signs of hepatic dysfunction. A liver biopsy revealed secondary amyloidosis.

Adult↗