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D Devictor

Publications and source records attributed to D Devictor.

At least 73 records · Page 4Linked to original sources

Diaphragmatic paralysis in children: diagnosis by TM-mode ultrasound.

Diaphragmatic paralysis, a difficult diagnosis in the pediatric age group, has classically been made by fluoroscopy or B-mode ultrasound. We report our experience with TM-mode exploration. Twenty-seven patients suspected to have diaphragmatic paralysis were examined by means of inspiratory and expiratory chest radiography, fluoroscopy and B-mode ultrasound. The diaphragmatic echo was recorded on TM-tracing during spontaneous breathing using coronal oblique scans. Direction, excursion and the pattern of the transition between inspiration and expiration were analysed. In 7 patients examination was normal and TM mode demonstrated movement of normal direction and excursion with a sharp aspect of the transition zone. Diaphragmatic paralysis was present in 11 patients: unilateral in 9 and bilateral in 2 cases. TM mode demonstrated paradoxical movement, reduced excursion and a smooth transition zone. In 9 patients with diaphragmatic dysfunction TM mode demonstrated movement in the normal direction but with reduced excursion and a smooth transition zone. Compared to other imaging modalities, TM-mode records diaphragmatic movements more objectively. It can identify direction of the movement even if they are fast and of weak amplitude and in the case of bilateral paralysis. TM can differentiate paralysis from dysfunction. Moreover, this low-cost, non-irradiating made of imaging can be performed at the bedside and is available on all basic devices.

Adolescent↗

Liver transplantation for end-stage liver disease associated with alpha-1-antitrypsin deficiency in children: pretransplant natural history, timing and results of transplantation.

Alpha-1-antitrypsin deficiency is an inborn metabolism error which can cause emphysema and liver disease. As regards the pathophysiology of liver disease, this deficiency is poorly understood, and it is also not known why only a small proportion of Pi ZZ individuals progress towards cirrhosis and liver failure. Since there is no specific therapy for end-stage liver disease associated with alpha-1-antitrypsin deficiency, patients are considered candidates for liver transplantation. In this paper, the natural history of 16 children who underwent liver transplantation is reviewed. Fourteen patients had neonatal cholestasis as a first symptom of the disease and hepatosplenomegaly was present in all children by the age of 12 months. In 11 children, jaundice recurred, always with liver function deterioration. Two patients had a histological paucity of interlobular bile ducts and required early transplantation due to rapid progression of liver failure. At the time of pretransplant assessment, all the patients in this study had portal hypertension and seven of them had experienced at least one episode of gastrointestinal bleeding. One child had moderate intrapulmonary shunts with hypoxemia, but the others had normal spirometry and blood gases. There was no other extrahepatic complication of alpha-1-antitrypsin deficiency. Eighteen orthotopic liver transplantations were performed in 16 patients. One patient died 8 days after retransplantation due to graft necrosis. Fifteen patients (94%) were alive after a median follow-up of 22 months with an excellent quality of life, normal serum alpha-1-antitrypsin levels and without evidence of liver disease recurrence or pulmonary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

Orthotopic liver transplantation in children with chronic liver disease and severe hypoxemia.

Liver transplantation has been considered until recently as an absolute contraindication in hypoxemic patients. We report our experience in nine patients who had orthotopic liver transplantation between June 1986 and June 1992. These patients had cirrhosis-related hypoxemia with intrapulmonary shunting (IPS). The arterial oxygen pressure (PaO2) on room air ranged from 47 to 78 mmHg. OLT resulted in resolution of hypoxemia and closure of IPS in five patients whose hypoxemia was higher than 60 mmHg, and in death for the remaining four patients who had severe hypoxemia (PaO2 < 60 mmHg). We conclude that hypoxemia is no longer a contraindication to liver transplantation. Patients having PaO2 levels higher than 60 mmHg should have OLT as soon as possible before reaching lower levels of PaO2, and combined lung-liver transplantation or heart-lung-liver transplantation should be discussed in patients with severe hypoxemia (PaO2 < 60 mmHg).

Adolescent↗

Fatal hemobilia after liver graft biopsy in a transplanted child.

Severe hemobilia after liver transplantation is a rare complication. We report one case of fatal hemobilia resulting from arterio-biliary fistula after a liver graft needle biopsy in a 7-year-old girl transplanted with a reduced-size graft. Arterial reconstruction was performed by an iliac conduit implanted in the aorta. The diagnosis of hemobilia was made by angiography but the hemorrhage could not be controlled by selective embolization of the bleeding arterial branch. The patient died from early and massive recurrent bleeding before surgery. The prevention of iatrogenic hemobilia and current therapeutic strategies are discussed.

Angiography↗

[Transplantation of reduced-size livers in children].

Nowadays, liver reduction techniques make it possible to use livers obtained from adults or adolescents for implantation in children. These techniques have been evaluated by analysis of 100 liver transplantations performed between January 1988 and October 1991 in 85 children. Forty-six full-size grafts implanted in 38 children (group 1) were compared with 54 reduced-size grafts implanted in 47 children (group 2). The overall actuarial survival at 4 years was 86 percent. There was no statistical significant difference between the two groups as regards the rates of death (8 versus 19 percent), reoperation (54 versus 64 percent), retransplantation (15 versus 16 percent), hepatic artery thrombosis (13 versus 15 percent) and graft survival (82 versus 70 percent) respectively. Haemorrhage was significantly more frequent in group 1 than in group 2 (P = 0.04), irrespective of whether transplantation was performed urgently or electively. Using reduced-size livers considerably increases the number of liver grafts available to children. Apart from a greater risk of haemorrhage, the results obtained with reduced-size livers were identical with those obtained with full-size livers.

Adolescent↗

Controlled liver splitting for transplantation in two recipients: technique, results and perspectives.

A technique of controlled liver splitting for transplantation in two recipients is proposed, based on a full anatomical assessment of the graft including arteriography and cholangiography on the back-table. Using eight livers, 16 patients received a graft: right liver (eight patients), left lobe (four) or left liver (four). Twelve patients required urgent or very urgent transplantation. Anatomical assessment of the graft demonstrated a portal bifurcation in all cases, a common trunk of the left and middle hepatic veins in five, a right biliary duplication in three and duplication of the left branch of the middle hepatic artery in one. After revascularization of the graft, bleeding was greater in patients with a right graft, particularly if the middle hepatic vein had been ligated. The main postoperative complications were hepatic artery thrombosis (four cases), biliary complications (four), portal vein thrombosis (two), haematoma (two) and abscess (two). No primary non-function of the graft was observed. The postoperative survival rate was 75 per cent. The four patients in whom transplantation was not considered urgent are still alive. The immediate survival rate of the grafts was 69 per cent. These results compare favourably with those in the literature. In spite of the technical, logistical and ethical problems raised by this technique, the results suggest that controlled liver splitting for transplantation in two recipients may in the future significantly improve the feasibility of liver transplantation.

Adolescent↗

Coma associated with intense bursts of abnormal movements and long-lasting cognitive disturbances: an acute encephalopathy of obscure origin.

We report six previously healthy children who several days after a prodromal illness had an acute encephalopathy that ran a biphasic course. It appears to constitute a recognizable syndrome with a good prognosis that can be differentiated from other encephalopathies of obscure origin as previously defined by Lyon et al. The active phase was dominated by coma or confusion and by abnormal movements, including disordered gesticulation and attacks of orofacial dyskinesia or limb dystonia associated with permanent rigidity and culminating in opisthotonic posturing. Repeated seizures were observed in only two patients. Permanent slow waves were recorded on the electroencephalogram in all patients, even during bursts of abnormal movements. Cerebrospinal fluid and results of serologic studies were normal throughout the course of the disease, and attempts at viral isolation and antiviral antibody detection yielded negative results. Brain imaging either showed no abnormalities or suggested a moderate degree of brain edema. The recovery phase, which extended for several weeks, was characterized by a rapid return of motor function and persistent behavioral and cognitive disturbances. Nonverbal reasoning recovered long before verbal expression returned to normal. Four patients eventually recovered fully, whereas two had mild sequelae.

Acute Disease↗

Enhanced tumor necrosis factor alpha in coronavirus but not in paracetamol-induced acute hepatic necrosis in mice.

Previous reports have demonstrated that tumor necrosis factor alpha (TNF-alpha) plays an important role in the pathogenesis of fulminant hepatic necrosis. The purpose of this experimental study was to measure TNF-alpha blood activity in paracetamol-induced liver necrosis and in coronavirus (MHV3)-induced fulminant hepatitis in mice. No elevation of TNF-alpha activity was found in hepatic failure complicating paracetamol poisoning. In contrast, TNF-alpha activity significantly increased in response to MHV3, reaching 16.3 +/- 5.5 U/ml from 24 h post infection (P less than 0.01). This augmentation was observed even though the virus was not detectable in the liver. Serum alanine aminotransferase levels were low and no histological lesion was observed. In conclusion, our study further supports the implication of TNF-alpha in virus-induced hepatitis failure and confirms that paracetamol poisoning does not cause increased TNF-alpha activity in the circulation.

Acetaminophen↗

[Cerebral complications of incontinentia pigmenti. A clinicopathological study of a case].

A baby born with the typical features of incontinentia pigmenti, developed convulsions from the 5th day onward. CSF was normal. CT scan showed diffuse hypodensities in the left hemisphere. The baby died at 2 months of age of primary pulmonary hypertension. The neuropathological examination showed a normal morpho-histogenesis of the brain. There were necrotic, non inflammatory changes in the cortex and white matter, which did not affect a vascular topography. The lesions appeared to have occurred in the neonatal period and were markedly predominating in the left hemisphere.

Abnormalities, Multiple↗

Emergency liver transplantation for fulminant liver failure in infants and children.

We report our results with orthotopic liver transplantation in children with fulminant liver failure. Thirty-five children with fulminant liver failure were evaluated for liver transplantation. The main causes of liver failure were viral hepatitis (54.2%), drug-induced liver injury (14.2%) and Wilson's disease (11.4%). Children were considered as candidates for liver transplantation only if hepatic encephalopathy was associated with a decrease in the level of factor V to below 25%. Seven children (20%) did not meet this criterion and recovered spontaneously. Six children (17.1%) had contraindications for liver transplantation and died. In three of these six children, contraindications included irreversible brain damage at the time of admission. Twenty-two children (62.8%) met the criteria for liver transplantation and were placed on the emergency transplant list. Three of them died awaiting grafts. Nineteen children underwent liver transplantation; 13 of them (68.4%) are alive without sequelae, after 6 mo to 4 yr of follow-up, at this writing. Four of the children who died after surgery had severe encephalopathy on admission that did not improve after liver transplantation. In conclusion, emergency liver transplantation appears to be an effective treatment for children with fulminant liver failure. Nevertheless, irreversible brain damage developed in 10 patients, and they died before or after surgery. We postulate that many of these deaths could have been avoided if children had been transferred to a liver transplantation facility and had undergone transplantation earlier. We emphasize that children with acute liver failure should be transferred to a center that performs liver transplantation before the development of hepatic encephalopathy.

Adolescent↗

Orthotopic liver transplantation with a reduced-size graft: an ideal compromise in pediatrics?

This study retrospectively reviewed the results of 81 orthotopic liver transplantations in 72 pediatric patients with either a full-size graft (n = 41) or a reduced-size graft (n = 40) during a 4-year period. Two types of liver reduction were used to obtain a left lobe graft (n = 30) or a left lateral segment graft (n = 10). The choice of the reduction technique was based on two criteria: the donor/recipient body weight ratio and the transverse size of the recipient's abdomen. The patients who underwent transplantation with a reduced-size graft were younger and smaller than those who received a full-size graft. The mortality rate of the candidates on the pediatric transplantation waiting list was 2.7% during the same period. The 3-year survival rate after elective transplantation was 85% and 75% in the full-size graft group and the reduced-size graft group, respectively (difference not significant). The use of reduced-size grafts was associated with higher amounts of red cell and fresh-frozen plasma transfusions after graft revascularization. The incidence of postoperative bleeding and bile leakage from the transected surface was low, whereas hepatic artery and biliary complications were less frequent in the reduced-size graft group than in the full-size graft group. At the time of follow-up, liver biochemical test results and quality of life were not different between the two groups. Despite the constraining technique and the increase in intraoperative blood loss, orthotopic liver transplantation with a reduced-size graft allows us to overcome the shortage of pediatric donors and improve the overall survival rate of children with end-stage liver disease.

Activities of Daily Living↗

Biliary complications after transplantation in children: role of imaging modalities.

Among a series of 140 liver transplantations in children, 21 biliary complications (BC) (15%) are reported. BC were identified from 2 days to 3 months after LT. Positive US findings were present in 20 cases. Cholangiography was obtained by opacification of a surgical drain in 3 cases, per-operatively in 3 and by PTC in 15. Drainage was placed in 9 and balloon dilatation performed in 2. Causes of BC include hepatic artery (HA) thrombosis in 7, HA stenosis in 1, anastomosis stricture in 7, anastomosis kink in 3, mucocele of cystic duct remnant in 2 and sludge in 1. Treatment was surgical in all, but 2 cases were treated percutaneously. There is a great difference in severity of prognosis between complications secondary to HA thrombosis and isolated BC. Role of US in diagnosis and of PTC and interventional radiology in treatment are emphasized.

Adolescent↗

Variability of clinical presentation of hepatic artery thrombosis in pediatric liver transplantation: role of imaging modalities.

Among a series of 90 pediatric liver transplantations, 9 cases of hepatic artery thrombosis (HAT) in 8 patients are reported. All cases were diagnosed in the first two weeks and confirmed angiographically and surgically. Clinical presentation was often unreliable with only 2 cases showing the typical pattern of massive hepatic necrosis. Five cases presented with biliary complications and in 2 cases, HAT was discovered fortuitously on duplex sonography. The role of imaging modalities is emphasized. Duplex sonography is the best non-invasive screening method and we recommend a routine daily examination in the first 2 weeks. CT is the most useful method to assess the extension of liver infarction. PTC remains necessary to evaluate biliary complications and to plan the best therapeutic approach in this devastating event. Two patients died, 2 had to be retransplanted (one patient twice) and are doing well, 2 are on a waiting list for retransplantation and one underwent an emergency surgical arterial desobstruction and is completely asymptomatic.

Child↗