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Biomedical subjects

D Devictor

Publications and source records attributed to D Devictor.

At least 55 records · Page 3Linked to original sources
PubMed1997

[Treatment of acute liver failure in children].

Among the main causes of acute liver failure (ALF) in children, metabolic diseases (especially in infants), viral and toxin or drug induced hepatitis are the most frequent. The cause remains, however, undetermined in about 30% of the cases. Management must be conducted in a pediatric hepatology unit or intensive care unit in relation with a pediatric transplant team in order: 1) to perform urgent etiological diagnosis; 2) to initiate specific therapy and symptomatic treatment; 3) to evaluate the severity and prognosis of liver disease for selection of children for emergency liver transplantation; 4) to evaluate contraindications to liver transplantation. The overall survival of post-emergency liver transplantation for ALF in children averages 65%.

Child↗
PubMed1997

[Agranulocytosis after application of silver sulfadiazine in a 2-month old infant].

BACKGROUND: A granulocytosis associated with the use of silver sulfadiazine has been described in burn patients. This agranulocytosis may be due to either an allergic reaction or a bone marrow toxicity by silver sulfadiazine. CASE REPORT: A 1-month old baby was hospitalized in pediatric intensive care unit after a stage one repair of a tracheoesophegeal fistula. Gastroesophageal reflux and tracheomalacia appeared in the post operative period. When she was 2-months old, she developed a perineal erythema. A topical therapy was begun, with daily applications of silver sulfadiazine and ketoconazole. Five days later, agranulocytosis (granulocytes = 0.21 x 10(9).L-1) occurred. Silver sulfadiazine therapy was immediately stopped. Granulocyte count returned to normal over a few days. No other etiology than silver sulfadiazine could be found to explain agranulocytosis. The small surface of administration, the chronology of the events and the rapid correction of the disorders after silver sulfadiazine interruption argued for an immuno-allergic reaction to this drug. Medical problems were easily controlled and the patient recovered well. CONCLUSION: This event occurred when sulfadiazine was applied in a small cutaneous area in a young baby. This may be an argument for an allergic reaction; alternatively, it could be explained by an increased cutaneous absorption of a cytotoxic drug in younger people.

Agranulocytosis↗
PubMed1997

Liver failure in children with hepatitis A.

There have been very few reports dealing with liver failure related to hepatitis A in children. Moreover, the criteria usually used for selecting patients with fulminant hepatitis A for liver transplantation have not been evaluated in children. Therefore, the current study was conducted retrospectively in a single French urban pediatric liver transplantation center to serve as a reminder of the potential severity of hepatitis A in children and to identify predictors of outcome. Children were selected by chart review using a data base system and were grouped according to outcome for analyses purposes. Over a 15-year period, 24 children with hepatitis A showed evidence of liver failure, including 6 children who did not develop hepatic encephalopathy, 7 children in whom encephalopathy occurred but resolved spontaneously, and 11 children in whom death or liver transplantation was the outcome. The mean age at onset was 6.5 years. Those with the most rapid onset of liver failure from onset of jaundice had the best chance of recovery without developing encephalopathy. Otherwise, no predictive factors of outcome were found at onset of liver failure. Among the 18 children who developed encephalopathy, the best early prognostic indicator of a poor outcome irrespective of the grade of encephalopathy, appeared to be a prothrombin time level below 21% of normal combined with a serum bilirubin level above 400 micromol/L. Therefore, these two prognostic indicators may be helpful in deciding liver transplantation in children with hepatitis A-induced fulminant liver failure.

Adolescent↗
PubMed1997

Urgent liver transplantation for biliary atresia: the experience in Bicêtre.

According to French rules for cadaver organ sharing, children with biliary atresia (BA) complicated with acute necrosis (ALN) can be registered on the waiting list for liver transplantation (LT) in a special intermediate grade urgent code. Over a 7 years period, 100 children have been submitted to elective LT for BA and 15 to urgent LT. Urgent procedures accounted for 25% of LT for BA in patients aged 0-2 years and 67% (8/12) in patients under 1 year of age. Children actuarial survival at 1, 12 and 48 months was respectively 66%, 60% and 60% versus 92%, 86% and 85%, deaths occurring earlier in the urgent group. Graft actuarial survival at 1, 12 and 48 months were 60%, 53% and 53% versus 85%, 77% and 76% (p < 0.05), respectively. Outcome of children and grafts after LT is not significantly different in BA cases and in other urgent indications, excluding retransplantations. In a LT program based on cadaver organ donation, allocation of in an urgent registration code to children with BA and ALN offers them more than 50% chance to escape death and does not result in wasting of grafts.

Actuarial Analysis↗
PubMed1996

[Fulminant hepatitis in children].

Viral hepatitis are one of the main causes of fulminant hepatic failure in children. Other causes of fulminant hepatic failure include metabolic diseases, especially in neonates and in infants, toxin-or drug-induced liver injury, hematologic and immune diseases. The spontaneous prognosis of fulminant hepatitis is poor with an overall mortality reaching 80%. The management of children with fulminant hepatic failure has been significantly transformed by emergency orthotopic liver transplantation. With this procedure, 1-year survival rates range from 55 to 80%. However, emergency liver transplantation still encounters serious problems including patient selection, organ availability, and the definition of optimum preoperative management of these patients. Several surgical innovations have been developed to resolve the shortage in liver grafts such as liver graft reduction or splitting for transplantation in two recipients. Because, spontaneous recovery remains unpredictable, new techniques are proposed such as the temporary orthotopic transplantation of auxiliary liver grafts, xenografts with monkey or pigs livers, hepatic assistance and bioartificial livers. These evolving strategies are currently under evaluation. Preventive treatment is one of the most effective approaches to fulminant hepatitis.

Age Factors↗
PubMed1996

How valid is emergency liver transplantation for acute liver necrosis in patients with multiple-organ failure?

Multiple-organ failure (MOF), defined as the failure of initially uninvolved organs, is the final step of definitive and massive liver necrosis. Emergency liver transplantation (ELT) has radically modified the outcome of acute liver failure and early primary graft failure, but the results of ELT in cases of MOF are unknown. From May 1988 to June 1993, 243 patients underwent a liver transplantation (LT). Thirty-seven patients (15.2%) who had an acute liver necrosis complicated by a MOF underwent an ELT. Twenty-one patients were children. An emergency retransplantation was performed in 16 patients. Three or 4 organ-system failures (OSF) were present in 13 patients. Before ELT, the mean Acute Physiology and Chronic Health Evaluation (APACHE) II score was 26.3 +/- 5.1. Six-month and 1-year survival rates were 37.8% and 25.9%, respectively, after ELT complicated by MOF, and 78% and 73.5%, respectively, in other cases of LT. Twenty-six patients had surgical complications (70%), whereas thirty-one patients had medical complications (84%). Twenty-two patients died during the postoperative period (60%). Before ELT, infection (P < .05), cardiovascular failure (P < .03), and more than two OSF (P < .05) were more frequent in patients who died after intervention. The APACHE II score (P < .05) and the length of stay in the intensive care unit before ELT (P < .05) were lower among survivors. In the context of liver allograft shortage, our results suggest that an ELT should not be performed in patients with cardiac failure, more than two OSF, or an APACHE II score higher than 30.

Acute Disease↗
PubMed1995

Liver transplantation in children with Alagille syndrome--a study of twelve cases.

Cholestasis associated with Alagille syndrome may, in a few cases, be extremely severe and result in major impairment in the quality of life during early childhood and end up in cirrhosis eventually. We report the results of liver transplantation in 12 children with a severe hepatic form of Alagille syndrome. All children presented with cholestatic jaundice from birth, peculiar facies, stenosis of the peripheral pulmonary artery, and posterior embryotoxon; butterfly-like vertebrae were present in 9 children. At the time of transplantation (mean age 7 years 10 months) refractory pruritus was present in 9 children, xanthoma in 11, and height and weight retardation in 11. Total serum bilirubin ranged from 116 to 322 mumol/L and total serum cholesterol from 3.5 to 29 mmol/L. Systolic right ventricular pressure was moderately raised (36 to 48 mmHg) in 5 children; mean creatinine clearance was 99 ml/min/1.73 m2. Histologic examination of the removed livers showed cirrhosis, severe annular fibrosis, and moderate portal fibrosis in 4 children each. Follow-up in the 11 survivors has ranged from 14 months to 5 1/2 years. All lead normal lives. Pruritus and xanthomas disappeared. Increase in height was observed in 8 of the 10 survivors who had growth retardation prior to transplantation. School level is normal in 4 (median age at LT: 5 yr 9 mo) and below normal in 6 (median age at OLT: 9 yr 9 mo). Liver function tests are normal in 10 children. Mean creatinine clearance is 101 ml/min/1.73 m2. These results indicate that the quality of life can be considerably improved after liver transplantation in children with a severe hepatic form of Alagille syndrome and suggest that it could be carried out before these children attend elementary school.

Alagille Syndrome↗
PubMed1995

Risk and prognostic factors of gut perforation after orthotopic liver transplantation for biliary atresia.

The aim of this study was to assess the risk and prognostic factors of gut perforation after orthotopic liver transplantation in children with biliary, atresia using univariate and stepwise regression analysis. Among 51 pediatric recipients who underwent transplantation because of biliary atresia after failure of portoenterostomy, 10 patients (20%) had 19 episodes of gut perforations after 14 transplantations. The median delay between transplantation and perforation was 13 days. These perforations were treated either by suture (n = 21) or ostomy (n = 11). The study of preoperative and perioperative variables showed that children with gut perforation were in surgery for a significantly longer period of time including a longer period of receiving hepatectomy and undergoing portal venous clamp. These children also needed large amounts of blood transfused during hepatectomy. After transplantation there was no difference regarding total steroid doses and early occurrence of cytomegalovirus disease between the two groups. Stepwise regression analysis identified three factors associated with the occurrence of gut perforation: duration of transplant operation, posttransplant intra-abdominal bleeding requiring reoperation, and early portal vein thrombosis. During the postoperative course, severe fungal infections were significantly more frequent in the gut perforation group. The 3-year patient survival rate was 70% in the group with gut perforation and was not different from the group without perforation (80%). This study shows that children with previous portoenterostomy carry a high risk of developing gut perforation after liver transplantation. This is especially true for those patients with the most difficult hepatectomies, which are responsible for the iatrogenic injury of the bowel. Other risk factors pointed out in this study were splanchnic congestion in case of prolonged portal venous clamp time or early portal vein thrombosis and repeated trauma of the bowel caused by reoperations. On the other hand, other well known risk factors, such as steroid therapy and viral diseases, were not involved in the occurrence of gut perforations in this study. Besides emergent surgical treatment, this type of complication requires aggressive therapy against fungal infections.

Biliary Atresia↗
PubMed1995

Nongalenic cerebral arteriovenous malformations in neonates and infants. Review of 26 consecutive cases (1982-1992).

We present 26 consecutive cases of nongalenic pial arteriovenous malformations (PAVMs) diagnosed in the neonatal period or in infancy. No diagnosis was made antenatally. Presenting symptoms in neonates were systemic cardiac manifestations (54%), seizures (31%), and hemorrhages (15%). In infants, hemorrhagic strokes and hydrodynamic disorders (external or internal hydrocephaly, macrocephaly, atrophy) both occurred in 38% of cases. Systemic cardiac manifestations and seizures were rare at that age (respectively 16% and 8%). Sixty-two percent of neonates and 31% of infants already had neurocognitive disorders (assessed by pediatric neurocognitive testing: Brunet-Leizine and Denver tests) when referred. The venous drainage and its anomalies (ectasias, stenoses, thromboses) were the main causes of symptoms. Atrophy and leukomalacic lesions occurred rapidly; they express local hydrovenous disorders and are specific to this population group. Untreated neonates and infants have a poor prognosis. Endovascular treatment, although partial and challenging in all instances, represents the treatment of choice in our series. Of the eight neonates treated, one improved to normal (12.5%), while four remained stable (50%): two neurologically normal, two with mild neurological deficit. Three (37%) died despite embolization (heart failure, multiorgan failure, postoperative death). Transient neurological complications occurred in two cases (25%): hemiparesis in one patient with a rolandic and in one with a thalamic AVM. Of the eight infants successfully embolized, one was significantly improved (12.5%) and is now neurologically normal, while five remained stable (62.5%): four neurologically normal, one with mild neurological deficit. One died between two sessions of embolization from intracerebral hemorrhage (12.5%). Hemianopsy occurred in one case (12.5%) after embolization of an occipital AVM. In one additional case in a normal child we failed to embolize the last small pial AVM of four after the three others had spontaneously thrombosed. With a minimal follow-up of 18 months and a maximum of 7 years, the review of our series shows 53% of the initial group of neonates and infants growing neurologically normal after therapeutic management in our institution; 23.5% died despite treatment, and the remaining 23.5% present minor neurological deficit. When targeted at the points of angioarchitectural weakness, embolization contributes to stabilizing a lesion. It should be undertaken rapidly to avoid loss of brain substance secondary to hemorrhage, atrophy, or leukomalacia, and to allow neurocognitive recovery and normal brain maturation. In our experience, these lesions are the most aggressive ones for the maturing brain, and the most difficult to approach technically. They represent a new therapeutic field and have their own specific anatomy and physiology.

Adolescent↗
PubMed1995

Flumazenil in the treatment of hepatic encephalopathy in children with fulminant liver failure.

OBJECTIVE: To evaluate the effect of flumazenil on hepatic encephalopathy complicating fulminant liver failure in children. DESIGN: Uncontrolled prospective study. SETTING: Pediatric Intensive Care Unit, tertiary care center. PATIENTS: 9 children with fulminant liver failure and hepatic encephalopathy awaiting emergency liver transplantation. INTERVENTIONS: Changes in hepatic encephalopathy grade and in electroencephalogram were recorded during the injection of a bolus of flumazenil (0.01 mg/kg/i.v. bolus) followed by a continuous infusion of flumazenil (0.01 mg/kg/h). MEASUREMENTS AND MAIN RESULTS: Before flumazenil, 7 children had grade 2 and 2 had grade 3 hepatic encephalopathy. Flumazenile injection mediated an arousal effect in 1 child in whom encephalopathy improved from grade 3 to grade 2. This effect lasted 30 min. No clinical response was observed in other children. An improvement of EEG anomalies was observed lasting 3 min in one child. Despite continuous infusion of flumazenil, encephalopathy worsened in all children. CONCLUSION: The effect of intravenous administration of flumazenil on hepatic encephalopathy in children with fulminant liver failure is inconsistent. Its efficacy is transient. The therapeutic value of flumazenil in children with fulminant liver failure awaiting a liver graft is minimal at this dosage.

Child↗
PubMed1995

Pulmonary arteriovenous shunting in children with liver disease.

Pulmonary arteriovenous shunting (PAVS) with hypoxemia is a severe complication of cirrhosis that may regress after liver transplantation. We report PAVS in 25 children with cirrhosis and in 1 with portal vein obstruction; proof of shunting was obtained by technetium Tc 99m microaggregated albumin pulmonary scanning or a high alveoloarterial O2 gradient or both. Cyanosis or dyspnea or both occurred at ages ranging from 6 months to 14 years, earlier in children with biliary atresia and polysplenia syndrome (p < 0.01). Mean arterial oxygen tension (PaO2) was 57 mm Hg (range, 42 to 81 mm Hg) during breathing of 21% O2 and 367 mm Hg (range, 179 to 535 mm Hg) in 100% O2. Cardiac index was always raised, significantly more in children with biliary atresia and polysplenia syndrome (p < 0.01). Seven untreated children died 3 months to 8 years after the diagnosis of PAVS. Eleven underwent liver transplantation: seven are alive (follow-up, 1 to 4 years) and have no signs of PAVS. The PaO2 value during breathing of 100% O2 was > 300 mm Hg in the survivors and < 200 mm Hg in the four nonsurvivors (p < 0.01). These results indicate (1) that PAVS can occur at any age in children with portal hypertension, and that the risk is highest and earliest in children with biliary atresia and polysplenia syndrome, (2) that early liver transplantation allows regression of PAVS, and (3) that the prognosis may in part be related to the level of PaO2 while the patient is breathing 100% O2. The results indicate that systematic screening for PAVS should be part of the examination of these children.

Adolescent↗