PubMed Health⌕ Search

Biomedical subjects

F Lacaille

Publications and source records attributed to F Lacaille.

At least 37 records · Page 2Linked to original sources
PubMed2000

Intestinal transplantation: indications, results and strategy.

The term 'intestinal failure' is now often used to describe gastrointestinal function insufficient to satisfy body nutrient and fluid requirements. The first recognized condition of intestinal failure was short bowel syndrome. Severe motility disorders such as chronic intestinal pseudo-obstruction syndrome in children as well as congenital intractable intestinal mucosa disorders are also forms of intestinal failure, because no curative treatment for these diseases is yet available. Parenteral nutrition and home parenteral nutrition remain the mainstay of therapy for intestinal failure, whether it is partial or total, provisional or permanent. However, some patients develop complications while receiving standard therapy for intestinal failure and are considered for intestinal transplantation. Indeed, recent advances in immunosuppressive treatment and the better monitoring and control of acute rejection have brought intestinal transplantation into the realm of standard treatment for intestinal failure. Although it has been used in humans for the past two decades, this procedure has had a slow learning curve. According to the current results, this challenging procedure may be performed in children or adults, only under certain conditions.

Adult↗
PubMed1999

Inflammatory pseudotumor of the liver: a rare benign tumor mimicking a malignancy.

We describe a 9-month-old boy in whom a hepatic tumor called angioma was diagnosed on ultrasonography, performed for abdominal pain. He was asymptomatic until 9 months later, when he presented with weight loss, jaundice, and a hard tumor in the left liver lobe. Radiological examination showed a calcified and heterogeneous tumor, amputation of the left portal vein, and dilatation of bile ducts, strongly suggesting malignancy. However, liver biopsy showed the typical findings of inflammatory pseudotumor, which are myofibroblastic cells and perivascular plasmocytes in a dense collagenous stroma. The tumor was surgically removed without local recurrence with a 2-year follow-up. Inflammatory pseudotumor in childhood is more common in the lung and elsewhere is often mistaken for a slowly growing cancer. This case points out the difficulties in the radiological evaluation of liver tumors in childhood and the importance of the histological differentiation of this lesion from malignancy before laparotomy for adequate indication of the treatment, which includes no medical treatment and surgery as conservative as possible.

Granuloma, Plasma Cell↗
PubMed1999

Liver transplantation in mitochondrial respiratory chain disorders.

UNLABELLED: Mitochondrial respiratory chain disease may lead to neonatal or late onset liver failure, requiring liver transplantation. In rare cases, the disease is restricted to the liver and the patient is cured after surgery. More frequently, other organs are simultaneously involved and neuromuscular or other extra-hepatic symptoms may pre-exist, or appear in the post-transplant follow up. Pre-transplant evaluation should aim to rule out neurological disease, which may be difficult to differentiate from signs accompanying liver insufficiency. Cerebrospinal fluid lactic acid levels, compared to blood lactate, may be suggestive of central nervous system involvement. Of 11 cases with respiratory chain disorders who had liver transplantation in various centres, 4 are alive and well on follow up, and 6 died, three of them having developed neurological disease post orthotopic liver transplantation. All three patients with initial liver and gastro-intestinal disease died early after transplantation, indicating that these may be poor candidates for this procedure. CONCLUSION: Liver transplantation is feasible in hepatic respiratory chain disorders, but extra-hepatic disease should be ruled out before transplantation. Extra-hepatic manifestations may, however, appear and cause patient death despite successful transplantation.

Electron Transport↗
PubMed1999

Up-to-date evolution of small bowel transplantation in children with intestinal failure.

PURPOSE: The aim of the authors was to report an up-to-date review of their experience with 26 intestinal transplantations in children since 1987. METHODS: A retrospective study was conducted of 26 patients with a mean age of 5 years (range, 0.3 to 14 years). Three groups were isolated. In group A (1987 to 1990), seven patients received nine isolated intestinal transplants for short bowel syndrome. Immunosuppression therapy consisted of cyclosporine, aziathioprine, and corticosteroids. In group B (1994-current), nine patients received nine isolated intestinal transplants for short bowel syndrom (n = 2), intestinal pseudoobstruction (n = 2), neonatal intractable diarrhea (n = 3), and Hirschsprung' disease (n = 1); hepatic biopsy results showed weak cholestasis or fibrosis. In group C (1994-current), 10 patients received 10 combined liver-small bowel transplants for short bowel syndrome (n = 3), neonatal intractable diarrhea (n = 4), and Hirschsprung' disease (n = 3); hepatic cirrhosis related to total parenteral nutrition (TPN) was shown in all cases. Groups B and C received immunosupressive treatment consisting of tacrolimus, aziathioprine, and corticosteroids. Posttransplant follow-up included intestinal biopsies of the small bowel twice a week and more frequently or combined with liver biopsy if rejection was suspected. RESULTS: Overall patient survival (PS) and graft survival (GS) are 61% (16 of 26) and 50% (13 of 26), respectively. In group A, severe intestinal allograft rejection occurred in six patients leading to graft removal (GS, 11%). Five patients died of TPN complications after graft removal (PS, 28%). One survivor is off TPN, and one currently is waiting for a second graft. In group B, six patients survived (PS, 66%). Causes of death include hepatic failure (n = 1), renal and liver failure (n = 1), and systemic infection (n = 1). Severe intestinal allograft rejection occurred in five patients, which neccessitated aggressive immunosuppression (antilymphocyte serum) leading to an incomplete functional recovery of the graft. Only two patients currently are off TPN. In group C, eight patients survived (PS, 80%) all of which are currently off TPN. One patient died during the procedure, and one died of severe systemic infection. Intestinal graft rejection occurred in six patients; rejection of the liver allograft occurred in five patients, yet all rejections were weak and successfully treated by corticosteroids (GS, 80%). CONCLUSIONS: Intestinal transplantation is a valid therapeutic option for children with definitive intestinal failure and not only for short bowel syndrome. Tacrolimus improves graft and patient survival (group A v group B). The lower severity of graft rejection in combined liver-small bowel transplantation improves functional results of intestinal transplantation in children without additional mortality or morbidity (group B vgroup C).

Adolescent↗
PubMed1999

Living-related liver transplantation in children: the 'Parisian' strategy to safely increase organ availability.

PURPOSE: The aim of the authors was to report their experience with living related liver transplantation (LRLT) in children, particularly focusing on the safety of the two-center "Parisian" strategy. METHODS: The records of donors and recipients of 26 pediatric living-related donor liver transplantations performed between November 1994 and March 1998 were reviewed retrospectively. Donors were assessed 1 year after transplantation for medical and overall status. RESULTS: Indications for LRLT included biliary atresia (n = 18), Byler's disease (n = 5), alpha-1-antitrypsin deficiency (n = 1), Alagille syndrome (n = 1), and undefined cirrhosis (n = 1). Liver harvesting consisted of either a complete left hepatectomy (n = 14) or left lateral hepatectomy (n = 12) without vascular clamping. The recipient procedure essentially was the same as in split liver transplantation. Mean overall cold ischemia time averaged 140 minutes (range, 90 to 230 minutes). Twenty-four of 26 patients had end-to-end vascular anastomoses without interposition. Biliary reconstruction consisted of a Roux-en-Y choledochojejunostomy in all patients. All recipients except one received cyclosporine A (CSA). Mean donor hospitalization was 8 days (range, 6 to 13) with normalization of all liver function assays by the time of discharge. There were no donor deaths and two postoperative complications (perihepatic fluid collection and bleeding from the wound). One year after donation, the initial 19 donors had resumed their pretransplant status. Two of the children who underwent transplant died. Thirteen of the recipients required reoperation for hepatic artery thrombosis (n = 2), portal vein thrombosis (n = 2), biliary complications (n = 6), fluid collection (n = 3), small bowel perforation (n = 1), and plication for diaphragmatic eventration (n = 1). With mean follow-up of 2 years, 24 of 26 patients are alive and well (patient and graft survival rate, 92%). CONCLUSIONS: LRLT is still controversial, even with minimal and decreasing donor risk. The "Parisian" strategy consists of harvesting the liver in an adult unit by an adult hepatic surgery team. The transplantation is then performed in a pediatric hospital by the pediatric liver transplantation team. The two steps of the procedure allow units specialized in adult surgery, on one hand, and pediatric liver transplantation, on the other hand, to dedicate themselves completely to their respective procedures, improving the safety of the harvest, and alleviating stress for both the medical staff and the families.

Adolescent↗
PubMed1999

Clinical utility of liver biopsy in children with acquired immunodeficiency syndrome.

BACKGROUND: Little is known about hepatic histology in children with AIDS, although the liver is frequently involved in the course of HIV infection. The clinical utility of liver biopsy in these patients is not well-defined. We reviewed retrospectively the results of this procedure in a group of infected children better to delineate its indications. PATIENTS AND METHODS: Eighteen children with AIDS underwent liver biopsy in our institution. The indications were unexplained fever in eight children, six of whom had an elevated erythrocyte sedimentation rate and clinical suspicion of mycobacterial infection; jaundice in four; suspicion of drug toxicity (dideoxyinosine) in two; discussion of treatment for chronic hepatitis B in three; suspicion of cytomegalovirus infection in one who had also AIDS cholangiopathy. RESULTS: Of the six children thought to have mycobacterial infection, two had the disease on biopsy, both of whom had abnormal liver enzymes. The children with unexplained fever had nonspecific findings, except for one with lymphoid interstitial pneumonitis who had a dense lymphoid infiltrate. Of the four with jaundice two had extensive necrosis caused by adenovirus infection in one and suspected herpes simplex infection in the other. The other two with jaundice had unexplained findings, severe necrosis and fibrosis in one case and hemophagocytosis in the other one; both improved clinically. Both children with suspected dideoxyinosine hepatotoxicity had nonspecific findings. The three children with chronic hepatitis B had mild lesions that were not an indication for treatment. CONCLUSIONS: Liver biopsy appeared to be useful in two groups of selected children with AIDS: when there is strong clinical suspicion of mycobacterial infection; and when the child is jaundiced.

Acquired Immunodeficiency Syndrome↗
PubMed1998

Crystalluria: a clinically useful investigation in children with primary hyperoxaluria post-transplantation.

Primary hyperoxaluria type I (PH I) is a congenital error of metabolism that can be manifested by an increased oxalate production, and ultimately result in kidney failure. After a combined liver/kidney transplantation, children with PH I have persistent excretion of oxalate that causes crystal formation in the urinary tract, and could result in systemic oxalosis and eventual graft failure. We speculated that crystalluria may be predictive of this nephrolithogenic tendency and thus investigated the effect of an intensive therapeutic strategy to prevent crystal formation in 13 children at our hospital. Oxalate crystal volume (OCV) measurements were performed at regular intervals for 36 months, and compared with urine supersaturation measurements. We found that crystalluria with the OCV measurement is non-invasive, easily performed, and gives feedback on the efficacy of PH I therapy within one hour. Further study is needed to determine whether this method is a better predictor of nephrocalcinosis than is supersaturation alone.

Adolescent↗
PubMed1998

Hepatitis C associated with Guillain-Barré syndrome.

Hepatitis C is frequently associated with immune-mediated diseases, such as cryoglobulinemia. Guillain-Barré syndrome is an acute demyelinating neuropathy of probable immune pathogenesis. We describe two patients with Guillain-Barré syndrome, and associated chronic hepatitis C, the second one previously treated with interferon. The link between both conditions may be hepatitis C being the trigger of this immune polyneuropathy. Guillain-Barré syndrome should be added to the list of conditions associated with hepatitis C.

Adolescent↗
PubMed1997

Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis.

OBJECTIVE: Several clinical trials of ursodeoxycholic acid (UDCA) have shown improvement of liver-function test results in cystic fibrosis (CF) with liver disease; however, there is no evidence that the long-term course will be affected. In view of the observations that UDCA can change the lipid profile and that patients with CF and liver disease are more likely to have essential fatty acid (EFA) deficiency, we elected to examine changes in the lipid profile and in the status of fat-soluble vitamins in response to UDCA. METHODS: Nineteen children with CF and liver dysfunction were recruited for a double-blind, crossover study of 1 year's duration, followed by treatment of the entire group. UDCA was administered at a dosage of 15 mg/kg per day, which, in the absence of a 50% decrease of alanine transaminase or aspartate transaminase or both within 2 months, was increased to 30 mg/kg per day. RESULTS: At entry, all patients had biochemical evidence of EFA deficiency. The lipid profiles during an average period of 25 months of follow-up showed a significant decrease in triglycerides (p <0.002), cholesterol (p <0.02), and total fatty acids (p <0.006). In addition, UDCA therapy led to an improvement in EFA status, as indicated by an increase (p <0.05) in the n-6 fatty acid concentration and a reduction (p <0.04) in the 20:3n-9/20:4n-6 fatty acid ratio. Although no change in vitamin E levels was observed, retinol metabolism was altered. There was an increase (p <0.02) in the unesterified retinol/retinol binding protein molar ratio in the absence of a difference in retinol binding protein concentration. Furthermore, retinyl esters, which normally account for less than 3% of circulating retinol, decreased (p <0.05) from 13.7% +/- 3.6% to 8.1% +/- 1.7%. CONCLUSIONS: This study confirms that UDCA alters lipoprotein metabolism and shows that it improves the EFA and retinol status of patients with CF and liver disease.

Adolescent↗
PubMed1996

[Pneumococcal meningitis in children: should probabilistic antibiotherapy of infectious meningitis be modified?].

BACKGROUND: Since a significant proportion of Streptococcus pneumoniae strains is now resistant to penicillin and sometimes to third-generation cephalosporin, it is necessary to reevaluate the initial therapy of bacterial meningitis proposed before identification of the organism and its susceptibility pattern. POPULATION: From 1 January 1992 to 31 March 1994, nine children with acute S pneumoniae meningitis were treated with ceftriaxone plus aminoglycoside as conventional initial therapy. Eight children were less than 1 year-old (five from 3 to 6 months). Five S pneumoniae strains were penicillin-resistant; four had a ceftriaxone minimal inhibitory concentration (MIC) of 0.047 to 0.094 mg/L and one of 1.5 mg/L. Ceftriaxone was given intravenously at doses of 50 mg/kg twice a day to patients less than 12 months old and 100 mg/kg once a day to patients older than 12 months. Intravenous amikacin (7.5 mg/kg twice daily) or netilmicin (3 mg/kg twice daily) were administered in combination. Dexomethasone was given to all children as adjunctive therapy. Follow-up lumbar puncture was performed after 24 to 36 hours of treatment. RESULTS: For each of the nine patients, cerebrospinal fluid was sterile with normal glucose level. After 2 or 4 days, initial therapy had been modified according to antibiogram and MIC. Monotherapy with ceftriaxone was continued in five children. Rifampicin was associated with initial bitherapy in one case. In two other patients, initial empiric therapy was stopped and changed to chloramphenicol. CONCLUSION: No case of bacteriological failure was noted in our patients but evolution of epidemiology and emergence of decreased penicillin sensibility in S pneumoniae strains (55% in our study) suggests that a third antibiotic (vancocin or rifampicin) should be associated with the standard first-line drug when S pneumoniae is suspected.

Adolescent↗