Individual distribution of muscle hypertrophy among hamstring muscle heads: Adding muscle volume where you need is not so simple.

PURPOSE: The aim of this study was to determine whether a 9-week resistance training program based on high load (HL) versus low load combined with blood flow restriction (LL-BFR) induced a similar (i) distribution of muscle hypertrophy among hamstring heads (semimembranosus, SM; semitendinosus, ST; and biceps femoris long head, BF) and (ii) magnitude of tendon hypertrophy of ST, using a parallel randomized controlled trial. METHODS: A total of 45 participants were randomly allocated to one of three groups: HL, LL-BFR, and control (CON). Both HL and LL-BFR performed a 9-week resistance training program composed of seated leg curl and stiff-leg deadlift exercises. Freehand 3D ultrasound was used to assess the changes in muscle and tendon volume. RESULTS: The increase in ST volume was greater in HL (26.5 ± 25.5%) compared to CON (p = 0.004). No difference was found between CON and LL-BFR for the ST muscle volume (p = 0.627). The change in SM muscle volume was greater for LL-BFR (21.6 ± 27.8%) compared to CON (p = 0.025). No difference was found between HL and CON for the SM muscle volume (p = 0.178).There was no change in BF muscle volume in LL-BFR (14.0 ± 16.5%; p = 0.436) compared to CON group. No difference was found between HL and CON for the BF muscle volume (p = 1.0). Regarding ST tendon volume, we did not report an effect of training regimens (p = 0.411). CONCLUSION: These results provide evidence that the HL program induced a selective hypertrophy of the ST while LL-BFR induced hypertrophy of SM. The magnitude of the selective hypertrophy observed within each group varied greatly between individuals. This finding suggests that it is very difficult to early determine the location of the hypertrophy among a muscle group.

Long-term outcome of liver transplantation in childhood: A study of 20-year survivors.

We report the results of a study of survival, liver and kidney functions, and growth with a median follow-up of 24 years following liver transplantation in childhood. From 1988 to 1993, 128 children underwent deceased donor liver transplantation (median age: 2.5 years). Twenty-year patient and graft survival rates were 79% and 64%, respectively. Raised serum aminotransferase and/or γ-glutamyl transferase activities were present in 42% of survivors after a single transplantation. Graft histology (35 patients) showed signs of chronic rejection in 11 and biliary obstruction in 5. Mean total fibrosis scores were 4.5/9 and 3/9 in patients with abnormal and normal serum liver tests, respectively. Glomerular filtration rate was <90 mL·min-1 in 35 survivors, including 4 in end-stage renal disease who were undergoing dialysis or had undergone renal transplantation. Median final heights were 159 cm for women and 172 cm for men; final height was below the target height in 37 patients. Twenty-year survival after childhood liver transplantation may be close to 80%, and final height is within the normal range for most patients. However, chronic kidney disease or altered liver biochemistries are present in over one third of patients, which is a matter of concern for the future.

The long-term outcome of hepatic artery thrombosis after liver transplantation in children: role of urgent revascularization.

Hepatic artery thrombosis (HAT), one of the most severe complications of pediatric orthotopic liver transplantation (OLT), often compromises graft and/or child survival. Of 590 OLT performed in 516 children over a 20-year period, 45 were complicated by early HAT, during the first 2 weeks after transplantation. Systematic Doppler ultrasonographic detection of HAT allowed successful surgical revascularization in 19 instances, resulting in a 20-year graft survival rate of 77% versus 24% of cases when revascularization was not attempted or failed. A combination of surgical emergency revascularization, biliary interventional radiology, biliary surgery and/or retransplantation resulted in an 80% 20-year patient survival rate, identical to that of transplanted children who did not experience early HAT. The majority of long-term survivors with their initial graft had normal liver tests, no biliary dilation on ultrasonography and minimal or moderate fibrosis on liver histology. A failed attempt at revascularization did not significantly alter patient survival. Despite these encouraging results, for the children and their parents to overcome the entire process in terms of reoperations, repeated radiological interventions, number of hospitalizations and emotional stress, remains an ordeal of such magnitude that it justifies renewed efforts to progress in the prevention of this complication.

Congenital portosystemic shunts in children: recognition, evaluation, and management.

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

Evaluation of FibroTest-ActiTest in children with chronic hepatitis C virus infection.

FibroTest-ActiTest (FT-AT) has been validated in adults with chronic hepatitis C virus (HCV) infection as a noninvasive alternative to liver biopsy (LB), but there are few data of its use in children. The objective of the present study was to evaluate FT-AT in children with HCV infection and to compare FT-AT analysis with liver histology. A total of 43 serum samples from 38 children with chronic HCV infection were analyzed retrospectively. Histological evaluation was performed according to the METAVIR scoring system. In 16 of the children, 21 serum samples were tested with FT-AT and compared to 21 LB (serum/LB pairs) in nontransplanted and liver-transplanted children. FT-AT was also measured in 22 infected children without LB and in 50 healthy controls. FT-AT values in controls were comparable to those of healthy adults, validating the adult FT-AT parameters in children. In most infected children (74%), the FT-AT score was

Bioavailability of oral vitamin E formulations in adult volunteers and children with chronic cholestasis or cystic fibrosis.

PURPOSE: To test bioequivalence of oral vitamin E formulations, water-soluble tocofersolan (test) and water-miscible (reference), in healthy adult volunteers, and their bioavailability in children with chronic cholestasis or cystic fibrosis. METHODS: In a two-way open randomized single dose cross-over design, 1200 IU were administered in 12 healthy volunteers and 100 IU/kg in 12 children with chronic cholestasis or cystic fibrosis. RESULTS: In healthy volunteers, formulations were not bioequivalent with a higher exposure to tocofersolan. In cholestatic children tocofersolan bioavailability was significantly higher than reference formulation (maximum plasma concentration: P = 0.008 and AUC: P = 0.0026). Bioavailability was not statistically different in cystic fibrosis. CONCLUSIONS: Oral tocofersolan was more bioavailable than the reference formulation in children with chronic cholestasis and similarly bioavailable in cystic fibrosis. Tocofersolan may represent an alternative to painful intramuscular vitamin E injections in chronic cholestasis, or to other oral formulations in cystic fibrosis.

Liver cell adenomas and portosystemic shunt.

We report the case of a young man who developed multiple liver cell adenomas 13 years after a mesentericocaval shunt. Radiological findings did not provide diagnosis. Histological findings of two biopsied nodules were compatible with liver cell adenoma. Our patient had no known risk factors for liver cell adenomas. We discuss the hypothesis that disturbed hepatic vascularisation could promote the development of liver cell adenomas.

[Screening for biliary atresia and stool colour: Method of colorimetric scale]. / Dépistage de l'atrésie des voies biliaires et couleur des selles : méthode de l'échelle colorimétrique.

Biliary atresia is a rare disease, but it is the major cause of neonatal cholestasis and the major indication for liver transplantation in children. Kasaï procedure can restore bile flow and prevent or slow progression of disease in a proportion of patients. Data show that the earlier the Kasaï procedure is performed, the better is the outcome. Therefore, rapid referral to an experienced centre, for prompt diagnosis and surgery, is strongly recommended. Unfortunately, the disease is often detected late, and Kasaï procedure is performed after 60 days of age. In an attempt to achieve earlier diagnosis and better outcome of Kasaï procedure, we propose to institute routine screening for biliary atresia using a stool colorimetric scale. This should help to identify earlier children who have acholic stools and may have biliary atresia. We postulate that this screening method will allow improving the results of the Kasaï procedure and providing children with the best chance of survival with their native liver.

Acute vanishing bile duct syndrome after ibuprofen therapy in a child.

We report the case of a 10 year-old girl who had Stevens-Johnson syndrome and cholestasis after ibuprofen therapy. Liver histology was compatible with vanishing bile duct syndrome. She received ursodeoxycholic acid, and liver tests normalized within 7 months. This report confirms that ibuprofen may induce acute vanishing bile duct syndrome.

Replicon typing of F18 fimbriae encoding plasmids of enterotoxigenic and verotoxigenic Escherichia coli strains from porcine postweaning diarrhoea and oedema disease.

The presence of fimbrial adhesin F18 is frequently found in enterotoxigenic Escherichia coli (ETEC) and verotoxigenic E. coli (VTEC) strains responsible for diarrhoea and oedema disease of weaned pigs. The F18 adhesin occurs in two antigenic variants: F18ab is characteristic of VTEC while F18ac is more typical for ETEC. F18 encoding plasmids of 17 phenotypically characterized porcine E. coli isolates (10 ETEC, 6 VTEC and 1 ETEC/VTEC) were tested with a DNA probe for F18 fimbrial adhesin and with replicon probes for the RepFIa, RepFIb and for the RepFIc family of basic replicons. In all the cases, the F18 probe hybridized to only one plasmid band of size higher than 42MDa. All F18 plasmids were determined to be unireplicon plasmids belonging to the RepFIc replicon family of the F incompatibility complex. There was no difference between F18ac plasmids of ETEC and F18ab plasmids of VTEC strains in terms of replicon type or subtype. However, the size of F18ab plasmids of the VTEC strains varied between 42 and 98MDa, in contrast to F18ac plasmids of ETEC strains (constantly approximately 98MDa).

Acute liver failure in infancy: a 14-year experience of a pediatric liver transplantation center.

Clinical charts of 80 infants younger than 1 year who presented over a 14-year period (1986 to 2000) with acute liver failure (ALF), defined as prolonged prothrombin time greater than 17 seconds and decrease of clotting factor V plasma level below 50% of normal, were reviewed retrospectively. The main causes of ALF were inherited metabolic disorders in 42.5% of cases, including mitochondrial respiratory chain disorders in 17, type I hereditary tyrosinemia in 12, and urea cycle disorders in 2; neonatal hemochromatosis in 16% of cases; and acute viral hepatitis in 15% of cases (hepatitis B in 6, herpes virus type 6 in 4, and herpes simplex virus type 1 in 2). The cause of ALF remained undetermined in 16% of cases. A total of 19 (24%) infants survived without orthotopic liver transplantation; 38 (47%) infants died from sepsis, multiple organ failure, or because the underlying disease contraindicated orthotopic liver transplantation (12 [15%] infants), and 23 (29%) infants underwent orthotopic liver transplantation within 12 months from onset, 12 of whom are alive with a mean follow-up period of 5.2 years from orthotopic liver transplantation. We conclude that ALF during the first year of life is a severe condition with poor prognosis, despite the advent of liver transplantation.

Role of multidrug resistance 3 deficiency in pediatric and adult liver disease: one gene for three diseases.

Class III multidrug resistance P-glycoproteins, mdr2 in mice and MDR3 in humans, are canalicular phospholipid translocators involved in biliary phospholipid (phosphatidylcholine) excretion. The role of an MDR3 gene defect in liver disease was initially suspected in a subtype of progressive familial intrahepatic cholestasis called PFIC3. Several MDR3 mutations have been identified in children with PFIC3 and are associated with a low level of phospholipids in bile, leading to a high biliary cholesterol saturation index. Mutations leading to a truncated protein are associated with an absence of canalicular MDR3 protein. The phenotypic spectrum of PFIC3 ranges from neonatal cholestasis to cirrhosis in young adults. There is now strong evidence that in addition to PFIC3, an MDR3 defect can be involved in intrahepatic cholestasis of pregnancy and in cholesterol gallstone disease. Therefore, at least three human liver diseases are due to a single gene deficiency. Patients with PFIC3 due to MDR3 deficiency may benefit from ursodeoxycholic acid therapy and could be good candidates for cell therapy in the future.

Genotypic and phenotypic characterization of potential virulence of intestinal avian Escherichia coli strains isolated in Algeria.

In order to characterize potential pathogenic Escherichia coli strains isolated from diarrheic hens and chickens originating from intensive battery rearing in North Algeria, the presence of a large range of virulence factors and markers was studied in 50 strains by DNA-DNA hybridization on colonies and phenotypic tests. The sequences we focused on were those coding for adhesins F5, F41, F17, Pap, Afa, and Sfa; intimin Eae; and toxins STa, STb, LT1, Stx1, Stx2, CNF1, and CNF2. The phenotypes explored were the colicins, aerobactin, hemolysins, and hemagglutinin production and serum resistance. The genotypic and phenotypic tests enabled us to categorize the isolates into two distinct groups: those with a potential to invade the host (27 strains were serum resistant and/or produced aerobactin), among which three strains were also potentially diarrheagenic, one strain was LT1 + F17+ Afa+ Pap+ (enterotoxigenic E. coli) and the two others were Stx1 (verotoxigenic E. coli). Twenty-three strains were colicinogenic, including 19 strains producing colicin V. This latter factor was also detected in isolates negative for the other virulence factors. On the basis of the type of erythrocytes agglutinated, we established 14 mannose-resistant hemagglutination patterns among the 37 strains tested, including 22 serum-resistant and/or aerobactin producing strains and 15 strains negative for these two characters. None of the strains produced alpha hemolysin, whereas two strains produced beta hemolysin and enterohemolysin, respectively. Congo red fixation was observed in 25 strains. No relationship could be detected between Congo red fixation and the presence of other virulence markers, such as serum resistance and aerobactin production. This study shows that among isolates originating from the feces of diarrheic chickens, the proportion of potentially diarrheagenic E. coli strains is low.

Characteristics of necrotoxigenic Escherichia coli isolated from septicemic and diarrheic calves between 1958 and 1970.

A total of 434 Escherichia coli isolated from septicemic calves between 1958 and 1965 and 430 E. coli isolated from diarrheic calves between 1967 and 1970 were studied by colony hybridisation and PCR assays for the presence of the cnf1- and the cnf2-like genes. They were also studied for the presence of genes coding for putative virulence factors associated with the CNF toxins including F17-, Pap- and Sfa-fimbrial adhesins and the recently described CDT-III toxin and AfaVIII-afimbrial adhesin. Thirty (7%) of the 434 septicemic strains were positive for CNF by colony hybridisation. Twenty-six were confirmed as necrotoxigenic E. coli type 2 (NTEC2) and four as NTEC1 by PCR. Thirty-five (8%) of the 430 diarrheic strains were positive for CNF by colony hybridisation. Five of them were studied by PCR and confirmed as NTEC1. The 26 septicemic NTEC2 strains and 20 of the 35 diarrheic NTEC including three of the five NTEC1 were positive for CDT-III. All adhesins studied were present in NTEC as well as in non-NTEC. NTEC1 were mainly Pap-, Sfa- and/or Afa8-positive, whereas NTEC2 were mainly F17- and/or Afa8-positive. This study shows that necrotoxigenic E. coli with their associated adhesins and toxins were present in calves as early as 1958, but their prevalence seems to have increased since that time.

Respiratory chain deficiency in Alpers syndrome.

Alpers syndrome is a progressive encephalopathy of early onset, characterized by rapid and severe developmental delay, intractable seizures and liver involvement in a previously healthy child. Here, we report on respiratory chain enzyme deficiency in the liver of four unrelated children presenting with epileptic encephalopathy and liver involvement diagnosed as Alpers syndrome. Interestingly, oxidative phosphorylation in skeletal muscle was normal in 4/4 and blood and CSF lactate in 3/4 patients. Liver involvement had a late clinical onset in patients with previously isolated epileptic encephalopathy. Based on these observations, we suggest 1. to give consideration to respiratory chain deficiency in the diagnosis of severe epileptic encephalopathy in childhood, even when no clinical or biological evidence of liver involvement or lactic acidosis is noted, and 2. to investigate the respiratory chain in a needle biopsy of the liver in children with epileptic encephalopathy prior to valproate administration if biochemical indications for respiratory chain disease or hepatic disturbance are noted, as this drug is believed to occasionally trigger hepatic failure and fatal outcome.