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Biomedical subjects

B Gridelli

Publications and source records attributed to B Gridelli.

At least 37 records · Page 2Linked to original sources
PubMed1998

Hodgkin's disease occurring in a child after liver transplantation.

Here we describe the case of a 14-year-old boy who underwent liver transplantation for post-Kasai biliary atresia when aged 4. Antirejection treatment consisted of prednisone and cyclosporine. At the age of 11 years the patient developed left cervical lymphadenopathy; the biopsy showed classical Hodgkin's disease(HD) of the mixed cellularity (MC) type. Neoplastic cells expressed CD30 and CD15, and were negative for CD45, CD20, CD3, CD43, and CD79a. Furthermore, they carried the EBV-related products LMP1 and EBER1/2. Treatment consisted of three cycles of adriamycin, bleomycin, vinblastine and DTIC (ABVD), followed by radiotherapy (2,000 cGys) on involved fields. At present, 42 months after the diagnosis of HD, the patient is still in complete remission. This is, to the best of our knowledge, the first reported case of classical HD following liver transplantation. The positivity of neoplastic cells for LMP1 and EBER1/2 indicates a possible role for immunosuppression in the development of the tumor, and whether a reduction in immunosuppression might have influenced the course of the disease is open to question.

Adolescent↗
PubMed1998

Steatocystoma multiplex and leuconychia in a child with Alagille syndrome.

Alagille syndrome is a rare autosomal dominant developmental disorder, characterized by congenital paucity of interlobular bile ducts, peculiar facies, posterior embryotoxon, bone abnormalities, and peripheral pulmonary artery stenosis. Cutaneous involvement in this disorder is quite rare and the association of Alagille syndrome with multiple comedones and cysts has been described only once. Here, we report the clinical and histological findings of a 7-year-old patient affected by Alagille syndrome who presented multiple cystic lesions and comedones reminiscent of steatocystoma multiplex and a congenital total true leuconychia. Although unexplained, the association of this syndrome with a developmental disorder of the pilosebaceous unit may not be fortuitous.

Alagille Syndrome↗
PubMed1997

Factors influencing malnutrition in children waiting for liver transplants.

Nutrition deficiencies are common in children with chronic liver disease. To determine whether age, hepatic dysfunction, or energy intake influences this malnutrition, we evaluated the nutritional status of 49 children aged 2.5 mo to 13 y (mean: 35 mo; median: 12 mo). The children were divided into two groups according to age: group 1-29 patients aged < or = 1 y (mean: 7 mo; median: 7 mo); and group 2-20 patients > 1 y (mean: 75 mo; median: 59 mo). Hepatic dysfunction was defined according to the Malatack criteria. Seventy-two-hour dietary intakes were recorded by a nutritionist. Nutritional status was assessed by anthropometric measures when the patients were enrolled on the waiting list for liver transplants. We evaluated the following indexes: weight, height, fat body mass, and lean body mass on the basis of height-age (age at which height reached 50th Italian height percentile). Mean height Z scores were low in both groups, but the difference was not significant. Mean weight Z scores and mean percentages of fat body mass were significantly lower (P < 0.001) in group 1 than in group 2 patients. In group 2, lean body mass and fat body mass were significantly lower (P < 0.05) in patients with moderate-to-severe hepatic failure than in patients with mild hepatic dysfunction. The mean energy intake was in the range of the recommended daily allowances for age but was insufficient for both groups of patients. The evidence of significant acute and chronic malnutrition confirmed the need for nutritional support, especially for younger and older children with moderate-to-severe hepatic dysfunction. We emphasize the necessity of accurate assessment of nutritional status by simple anthropometric measurements to be sure of the effects and adequacy of the nutritional intervention.

Adolescent↗
PubMed1996

Hepatitis C virus genotypes and reinfection of the graft during long-term follow-up in 35 liver transplant recipients.

To understand the clinical outcome of hepatitis C virus (HCV) recurrence, data from 35 liver transplant recipients who survived more than 6 months were reviewed. The presence of HCV-RNA was evaluated and genotyping was performed. On the basis of alanine aminotransferase (ALT) levels, patients were sorted into four groups. In 20 patients, a chronic elevation in ALT was found; HCV-RNA detection was positive in 17/17 and the following genotypes were found in 15 of them: 1b in ten patients, 2a in four patients, and 3a in one patient. In 11 patients, ALT levels remained normal throughout follow-up; in nine of them HCV-RNA was positive; HCV genotyping was available in eight patients and identified type 1b in two, type 2a in five, and type 3a in another patient. In two patients, ALT fluctuated above and below the upper limits of normality; type 1b HCV-RNA was found in one of them. In two patients, after an initial period of normality, ALT levels showed an abrupt rise; HCV-RNA was positive and type 1b was identified in both patients. Eight patients developed HCV-related deep jaundice and three of them spontaneously recovered. Progressive hepatic injury occurred in eight patients, six with chronic ALT elevation and two showing a late ALT elevation; genotype 1b was present in seven patients while in one, genotype 3a was found; sub-acute graft failure developed in five of them, leading to death in two and retransplantation in the others; the other three patients are alive with recurrent overt cirrhosis. The 1, 3, and 5 year actuarial survivals were 89%, 79%, and 63% respectively. The 1, 3, and 5 year actuarial risks of progressive graft damage were 6%, 7%, and 15%, respectively. In conclusion, HCV reinfection causes a slow decrease in the long-term patients' survival. Persistent elevation of ALT is more frequently observed in patients with genotype 1b infection.

Adolescent↗
PubMed1996

Expression of lysosomal acid lipase mutants detected in three patients with cholesteryl ester storage disease.

Lysosomal acid lipase (LAL) gene mutations were identified in three patients with cholesteryl ester storage disease (CESD). Direct sequencing of genomic DNA revealed that: patient 1 was a compound heterozygote for a P181L mutation and an A to G3' splice site substitution that causes skipping of exon 7, with a loss of 49 amino acids from LAL (delta 205-253); patient 2 was a compound heterozygote for a G66V mutation and a 5' splice site mutation (G to A) that leads to skipping of exon 8 (delta 254-277); and patient 3 was a compound heterozygote for a L273S mutation and an unidentified null allele. Furthermore, patients 2 and 3 showed a novel G-2A polymorphism that could be detected by an Xbal restriction fragment length polymorphism. All these mutants and a previously reported H274Y allele were expressed in vitro in HeLa cells using the vaccinia T7 expression system. The resulting recombinant proteins were inactive towards cholesteryl oleate and trioleylglycerol, demonstrating the direct involvement of these mutations in the pathogenesis of CESD. Immunoblotting of normal LAL expressed in HeLa cells revealed four major molecular forms, at least two of high molecular mass (54 and 50-51 kDa) and two of low molecular mass (42 and 43 kDa). L273S and P181L substitutions and delta 254-277 were shown to result in altered LAL molecular forms, some of which suggest that post-translational processing may interfere with the catalytic activity of LAL.

Child↗
PubMed1996

Paediatric liver transplantation in Italy.

The paediatric liver transplantation programme was started, at our institution, in 1988 and since then 75 children have received 79 transplants, which corresponds to about 85% of all the paediatric liver transplantations performed in Italy. The main indications to transplantation were biliary atresia, familiar cholangiopathies and metabolic diseases. The patients came from all the regions of Italy, except for 2 of them who came from abroad. Patients with malnutrition and impaired growth underwent an aggressive nutritional programme whilst waiting for transplantation. The transplants were performed using whole grafts, reduced size grafts and, recently, with grafts from split livers, in 7 cases. Immunosuppression was based on a double drug therapy with Cyclosporine and low dose steroids. Steroid resistant acute rejection was treated, with OKT3 until 1993 and with conversion from Cyclosporine to FK506, thereafter. Interventional radiology was most useful in treating biliary complications. The introduction of Gancyclovir greatly reduced the incidence and severity of cytomegavirus related complications. Three years survival after liver transplantation was 62% for children under 2 years of age and 78% for those between 2 and 15 years. These results do not differ substantially from those reported by the European Liver Transplant Registry.

Adolescent↗
PubMed1995

Early metabolic treatment after liver transplant: amino acid tolerance.

OBJECTIVE: We investigated the amino acid (AA) tolerance during Total Parenteral Nutrition (TPN) in adult patients undergone liver transplant (LTX). DESIGN: The treatment (Glucose and AA), induced on the 2nd postoperative day, was later maintained with 27 kcal/kg Ideal Body Weight (IBW) as glucose and 0.12 (12 patients: protocol #1), 0.18 (10 patients: protocol #2) and 0.25 g nitrogen (N)/kg IBW (13 patients: protocol #3) till end of the 6th postoperative day. The N intake was sequentially modified in protocol #2 and #3 to increase the supply of the amino acid (AA) that resulted in an infusion plasma level below the expected "normal" range (between 1 and 1.6 times the overnight fasting plasma level of volunteer). PATIENTS: 35 consecutive adult patients without diabetes and organ failures for the entire study period. MEASUREMENTS: Plasma AA profile was measured before LTX and at the last TPN day under continuous infusion. During #1 and #2 protocol, many AA resulted below or at the lower range of the norm while, during 0.25 gN/kg IBW infusion, the majority of the administered AA significantly increased with respect to reference values. Nevertheless, they remained in the "normal" plasma range indicating that they were supplied in an optimal amount (particularly the aromatic and sulphurated ones, potentially toxic if liver function is impaired, and the branched chain AA (BCAA) given at consistent dosage: 0.5 g/kg). Arginine resulted significantly increased (Arg: 1.9 times the reference) and cystine (Cys: 0.45), serine (Ser: 0.8) and taurine (Tau: 0.85) remained significantly lower than "normal" as well as the not administered citrulline (Cit: 0.58) and alfa amino butyric acid (Aba: 0.41). The AA (and calorie) load almost balanced the N losses during the 5th (0.411 +/- 0.038) and 6th study day (0.305 +/- 0.019 gN/kg). CONCLUSIONS: 0.25 gN/kg could be considered the minimum N load in the uncomplicated adult LTX recipients, for reassuring a balanced plasma AA pattern and body N turnover in the early postoperative phase.

Adolescent↗
PubMed1994

Sensitivity of baboon lymphocytes to cyclosporin A and FK 506: relative resistance of alloactivated cells to CyA.

The interspecies differences in CyA pharmacokinetics necessitate the establishment of optimal immunosuppressive doses in the baboon, especially as its use as host for preclinical xenografts is anticipated. We assessed the immunosuppressive effects of CyA and FK 506 on lymphocytes from chacma baboons, using human cells for comparison. At concentrations up to 100 mumol/l, neither drug was toxic to lymphocytes. FK 506 inhibited baboon and human lymphocyte proliferation and IL-2 synthesis equally. In contrast, approximately four times higher doses of CyA were needed to inhibit baboon lymphocytes responding to alloantigens. This may explain the inadequate immunosuppression of baboon graft recipients treated with clinically acceptable doses of CyA. We propose that CyA whole blood target levels of +/- 1500 ng/ml should be used in this species and we provide evidence that chacma baboons are able to tolerate such doses without nephrotoxicity.

Animals↗
PubMed1994

Prognostic value of the activated partial thromboplastin time after orthotopic liver transplantation. A prospective study.

In this prospective study, we evaluated the predictive value of activated partial thromboplastin time on day 8 post transplantation for event-free survival in patients who had had orthotopic liver transplants; both death and retransplantation within 6 months were the events considered. In a 4-year period, 109 patients had orthotopic liver transplants in our hospital, and 104 were eligible for the study since they survived and were not given new transplants within 8 days. The activated partial thromboplastin time was significantly longer in patients who survived event-free for less than 6 months than in those with longer event-free survivals. Kaplan-Meier curves showed that patients with normal activated partial thromboplastin times were nine times more likely to survive more than 6 months without events than patients with prolonged values. The positive predictive value of activated partial thromboplastin time for event-free survival was 88% and the negative predictive value was 54%, indicating that the test is useful for predicting patient outcome. We suggest that activated partial thromboplastin time be performed on day 8 post transplantation to predict the medium-term event-free survival.

Adolescent↗