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Biomedical subjects

F Lacaille

Publications and source records attributed to F Lacaille.

At least 19 recordsLinked to original sources
PubMed2006

Severe dysimmune cytopenia in children treated with tacrolimus after organ transplantation.

Rare cases of dysimmune phenomena after solid organ transplantation were described in the past. In the present series, we describe six children who developed severe dysimmune anemia or thrombocytopenia while treated with tacrolimus after liver or small bowel transplantation. All patients were off steroids or under low doses alternate day steroid medication when dysimmune cytopenia developed. All patients had positive anti-platelets antibodies and/or Coombs' positive anemia. Therapy was successful in all six patients with a rapid response to corticosteroids in three children, and to anti-CD20 monoclonal antibodies (rituximab) in the three others. The pathogenesis of these rare dysimmune/autoimmune disorders might be related to the interference of tacrolimus with T-cell functions and/or the endogenous control mechanisms of T-lymphocyte activation and down-regulation. Although rare, these complications must be known when discussing protocols of immunosuppression.

Anemia↗
PubMed2004

[Hereditary cholestasis, an unusual etiology of pruritus in the infant].

INTRODUCTION: Pruritus in the infant is predominantly related to common dermatosis. General causes remain exceptional. We report two cases of pruritus in infants revealing anicteric cholestasis. OBSERVATIONS: Case no 1. A thirteen month-old boy had exhibited pruritus since the age of 2 months. The clinical examination was non-specific. Biological explorations revealed an isolated and moderate rise in total bilary acids. The search for mutations in the genes of a familial fibrogenic cholestasis was negative. The diagnosis retained was hypercholanemia. Treatment combined ursodesoxycholic acid and rifampicine, which controlled the pruritus and normalized the bilary acid levels. Case no 2. A twenty-one month-old boy had exhibited pruritus since the age of 2 months and delayed growth. The clinical examination was unspecific. The biological explorations revealed cholestasis with normal delta GT, moderate cytolysis and liposoluble vitamin deficiency. The hepatic biopsy was normal. The diagnosis retained was familial fibrogenic cholestasis. Treatment combined ursodesoxycholic acid and rifampicine, which controlled the pruritus and normalized the hepatic parameters. DISCUSSION: Non-dermatological isolated pruritus is rare in infants. These two observations illustrate two abnormalities in bilary acid transport. Hypercholanemia is a faulty canalization of bilary acids by the hepatocyte. Familial fibrogenic cholestasis is a default in the elimination of these bilary acids. Such pathologies must be evoked because specific treatment will treat the symptoms and avoid the evolution of familial fibrogenic cholestasis towards cirrhosis.

Cholestasis↗
PubMed2004

Magnetic resonance imaging for the diagnosis of acute leukoencephalopathy in children treated with tacrolimus.

The leukoencephalopathy induced by tacrolimus is increasingly recognised as an important cause of neurological complications after transplantation. Magnetic resonance imaging (MRI) is of major help in the differential diagnosis of infection or vascular injury. We describe two children with coma and seizures after transplantation, in whom the results of MRI with FLAIR (fluid-attenuated inversion-recovery) and DWI (diffusion-weighted images) were the main positive elements for the diagnosis of drug-induced toxicity. The results of DWI favoured the role of oedema and/or demyelination in the pathophysiology of this disorder. Unlike other reported patients, in whom all symptoms resolved, lesions persisted, albeit improved, on the control MRI, and both children demonstrated learning disabilities after several years of follow-up.

Acute Disease↗
PubMed2002

[Chronic hepatitis C virus infection in children].

Peripartum transmission is today the main cause of hepatitis C virus (HCV) infection in children. HCV infection rarely causes a clinical illness during childhood and adolescence, except when it is associated with additional risk factors such as hepatitis B or HIV infection, chemotherapy or immunodeficiency. Present data suggest that between 20 to 50 percent of contaminated infants will become spontaneously non viremic within 15 to 20 years. Studies on treatment with interferon-alpha are limited and show a mean recovery rate of 40%.

Adolescent↗
PubMed2001

Long term results of liver-kidney transplantation in children with primary hyperoxaluria.

From 1990 to 2000, we performed eight liver-kidney transplants in eight children, aged 1-16 years, with end-stage renal failure (ESRF) due to primary hyperoxaluria (PH1). The duration of dialysis before transplantation ranged from 2 to 42 months (mean 14 months) and was <1 year in four patients. Only the first patient underwent postoperative hemodialysis; in the other five, we chose to induce maximal diuresis from the first hours with intravenous and intragastric hyperhydration (> or =3 l/m2 per day). High water intake with nocturnal tube hydration was maintained for 6 months to 5 years, as long as oxaluria exceeded 0.5 mmol/day. A quadruple sequential immunosuppressive regimen was used. Two patients died during liver graft surgery. The other six patients are alive and well, with a mean follow-up of 7.4 years (range 5-11 years). Patient and graft survival is 75% at 5 years. At latest follow-up, liver tests were normal in all six patients; creatinine clearance ranged from 55 to 95 ml/min per 1.73 m2 (mean=74). Oxaluria was lower than 0.4 mmol/day in all patients (mean=0.22). The six patients underwent 15 renal biopsies, 1-11 years after transplantation. Chronic transplant nephropathy was present in four patients and mild cyclosporin nephrotoxicity in another. No oxalate crystals were seen and repeat ultrasonography has been consistently normal in all patients. The three patients with bone oxalosis showed progressive complete healing of bone lesions. All six children or adolescents now live a normal life. From this series, we conclude that early combined liver-kidney transplantation is the treatment of choice for children with ESRF due to primary hyperoxaluria.

Adolescent↗
PubMed2001

[Differential diagnosis of elevated transaminases].

Elevated serum levels of transaminases must always be considered as an abnormal finding. Drugs and toxins must be eliminated first as possible hepatotoxic agents or co-factors. Antiviral hepatitis A IgM determination is the first test to perform. However, other viruses with spontaneous benign courses are the most frequent cause. Only if the initial presentation is severe, or if liver tests remain abnormal after several weeks, can other rare diseases be sought. Liver biopsy is rarely necessary to reach a diagnosis.

Biopsy↗
PubMed2001

Apolipoprotein B Arg3500Gln mutation prevalence in children with hypercholesterolemia: a French multicenter study.

BACKGROUND: Familial defective apolipoprotein B-100, a dominantly inherited form of hypercholesterolemia caused by a single Arg3500Gln mutation, is silent in childhood but may confer a high risk of cardiovascular disease in adulthood. The objective was to determine the prevalence of familial defective apolipoprotein B-100 in hypercholesterolemic French children and to provide a basis for targeting screening efforts in this population. METHODS: One hundred ninety children attending 13 pediatric clinics distributed throughout France were included based on the presence of type IIa hypercholesterolemia with a plasma low-density lipoprotein-cholesterol level of more than 130 mg/dL. The Arg3500Gln mutation was detected in dried blood spots using a polymerase chain reaction assay combined with enzymatic restriction. RESULTS: Three hyperlipidemia phenotypes were found: monogenic dominant pure hypercholesterolemia (n = 117), polygenic hypercholesterolemia (n = 43), and combined hyperlipidemia (n = 11). Three unrelated children were heterozygous for the Arg3500Gln mutation; all three had monogenic dominant pure hypercholesterolemia (3/94 families; 3.2%), yielding a prevalence of 1.83% (3/164) in hypercholesterolemic children, which is similar to prevalences reported in European adults. CONCLUSIONS: The familial defective apolipoprotein B-100 mutation was common (1/31) in children with a phenotype of familial hypercholesterolemia, supporting screening in this population with the goal of preventing premature cardiovascular events.

Adolescent↗