Gilbert Huault

Intrathecal synthesis of different alpha‐interferons in patients with various neurological diseases

ABSTRACT – CSF and sera from 238 newborns and children with various neurological diseases were assayed on bovine cells for the presence of alpha‐interferon (IFN). An intrathecal synthesis of pH 2‐resistant alpha‐IFN was recovered in all newborns and in more than 90% of children with herpes encephalitis. It was also observed in one case of mumps encephalitis and in one case of encephalitis associated with Influenza A infection. An acid‐labile alpha‐IFN production was detected in CSF from more than one half of patients with viral meningitis or active congenital rubella and in those with neurological complications of systemic lupus erythematosus. This alpha‐IFN subtype was also detected in CSF from only 2/37 children with measles encephalitis. In contrast, no alpha‐IFN (<2 IU) in CSF was found among patients with subacute sclerosing panencephalitis, Guillain‐Barré syndrome, Reyes syndrome, acute cerebellar ataxia, infantile spasms or facial paralysis of unknown origin.

Acute transverse myelitis in children: Clinical course and prognostic factors

The objective of this study was to describe the clinical course of acute transverse myelitis in children, to identify prognostic factors, and to compare our findings with published data. Twenty-four children, aged 2 to 14 years and admitted with a diagnosis of acute transverse myelitis, were studied. Clinical features and results of investigations were collected at admission and during the course of the disease. Motor, sphincter, and global outcomes were compared with those in the main adult and pediatric series. During the initial phase, the most common presenting symptoms were pain (88%) and fever (58%). Motor loss preceded sphincter dysfunction in two thirds of patients and became bilateral in half of the patients. When maximal deficit was achieved (plateau), the patients presented a combination of sensory, motor, and sphincter dysfunctions without radicular involvement. The motor loss consistently involved the lower limbs but was inconsistent and moderate in the upper limbs. The mean duration of the plateau was 1 week. The recovery phase was characterized by a progressive improvement of all deficits. Sphincter dysfunction improved more slowly than did the other deficits. A full recovery was achieved by 31% of the patients; minimal sequelae were present in 25% and mild to severe sequelae in 44%. An unfavorable outcome was associated with complete paraplegia (P = .03) and/or a time to maximal deficit shorter than 24 hours (P = .005). A favorable outcome was associated with a plateau shorter than 8 days ( P = .03), the presence of supraspinal symptoms (P = .01), and a time to independent walking shorter than 1 month (P = .01). The course of acute transverse myelitis in children proceeds through three stages, an initial phase, a plateau, and a recovery phase, each characterized by specific clinical features. The global outcome was favorable in 56% of patients. Several prognostic factors were identified. (J Child Neurol 2003;18:401—406).

High dose methylprednisolone in severe acute transverse myelopathy

No effective treatment has been shown for patients with acute transverse myelopathy. In an open study five children with severe acute transverse myelopathy were treated with intravenous methylprednisolone and compared with a historical group of 10 patients. The results show that in the methylprednisolone treatment group compared with the historical group of 10 patients: the median time to walk independently was significantly reduced (23 v 97 days); the proportion of patients with a full recovery within 12 months was significantly higher (80 v 10%); all patients had complete motor recovery within one year in contrast with only two of 10 patients in the historical group; and serious adverse effects did not occur. This pilot study suggests that high dose methylprednisolone is effective in the treatment of acute transverse myelopathy.

Peliosis hepatis with initial presentation as acute hepatic failure and intraperitoneal hemorrhage in children

Peliosis hepatis, a condition characterized by the presence of blood-filled lacunar spaces in the liver, usually has a chronic presentation pattern and is mainly reported in adult patients in association with chronic wasting disorders and after administration of various drugs. The present report concerns two previously healthy young children in whom peliosis hepatis initially presented as acute hepatic failure and who had Escherichia coli pyelonephritis. Both patients had active intraperitoneal hemorrhage from the peliotic liver lesions, and liver ultrasonography showed multiple hypoechoic areas of different sizes, which in this context should suggest the diagnosis. One child died from hypovolemic shock and the other recovered. This study indicates that acute peliosis hepatis can be a serious life-threatening disease in children.

Outcome of ABO-incompatible liver transplantation in children with no specific alloantibodies at the time of transplantation.

The shortage of suitable liver donors for children has motivated the use of ABO-incompatible (ABO-I) grafts for transplantation in urgent situations. However, survival after ABO-I liver grafts has been reported at about 30% as compared with 80% in cases of ABO-identical or -compatible liver grafts. This difference has been attributed to antibody-mediated, hyperacute or chronic liver rejection, due to preformed ABO antibodies (alloantibodies). In this study, we report our results with ABO-I livers in children without alloantibodies at the time of transplantation. From January 1988 to June 1993,143 OLT were performed in 122 children. Eight children received 8 ABO-I liver grafts. Of these, 7 patients were included in the study. All 7 were alloantibody free before OLT. Five children were spontaneously alloantibody free, while in 2 children, the plasma alloantibodies were eliminated before and after transplantation using intravenous infusion of specific blood group antigens of the donor blood group (soluble antigens). Immunosuppression consisted of a triple-drug treatment combining CsA, AZA, and steroids. The follow-up period was between 10 and 48 months. One child died from a surgical complication. Six children survived, but 1 died 10 months later from intestinal obstruction. There were no graft losses and no episodes of hyperacute or chronic rejection. The graft and patient survival rate was 71%. There was a 28% incidence of rejection, but all were mild (requiring steroid boluses only), Our results suggest that the absence of ABO alloantibodies at the time of and after transplantation can protect ABO-I liver grafts against antibody-mediated rejection, whether hyperacute or chronic, and that soluble antigens are effective in eliminating alloantibodies in children.

Paralytic poliomyelitis in vaccinated children

Paralytic poliomyelitis were observed in two healthy children who both had previously received four doses of standard inactivated poliovaccine (IPV). These children, whose immune defenses were normal, failed to respond to IPV. This absence of antibody response might be related to an insufficient antigenicity of inactivated vaccines, justifying the extensive use of the more potent IPV, now produced on continuous cell lines.