[A case of hereditary sensory and autonomic neuropathy type IV diagnosed following the development of acute encephalopathy due to heat stroke].

A fourteen-month-old girl, who had shown remittent fever frequently from the neonatal period, hypohidrosis, frequent change of face color and self-mutilation of the 1st and 2nd fingers of both hands and tongue in the first months of her life, developed an acute encephalopathy with generalized tonic convulsion outdoors on a sunny hot day. Generalized tonic convulsion subsided within two days, but doll's eye phenomenon, loss of pupillary reaction to light, palpebral myoclonus, and ballismus of arms and legs followed L-dopa showed some effect on the ballismus 1 month after the attack. During the hospital stay, biopsy of sural nerve was performed. Morphometric and ultrastructural studies of the sural nerve demonstrated decreased numbers of unmyelinated and small myelinated fibers. Skin biopsy of the leg revealed sweat glands with no nerve terminals, axons and Schwann cells around them. She was diagnosed as having hereditary sensory and autonomic neuropathy type IV based on the histological and clinical findings. After discharge, bone fracture was found three times without any evidence of trauma. Acute encephalopathy, probably produced in relation to the underlying neuropathy, was considered to be due to heat stroke.

Human herpesvirus 7 infection associated with central nervous system manifestations.

The clinical features of infection with human herpesvirus 7 (HHV-7) are not well described. Exanthem subitum is the only illness that is confirmed to be caused by HHV-7. We report two children who had exanthem subitum associated with central nervous system manifestations. Two strains of HHV-7 were isolated sequentially from peripheral blood mononuclear cells and saliva of the some child who had exanthem subitum complicated with acute hemiplegia in childhood. Two strains were confirmed to be HHV-7 by means of monoclonal antibodies to human herpesvirus 6 (HHV-6) and HHV-7, polymerase chain reaction, and DNA analysis. During the convalescent period, the antibody titer to HHV-7 rose from less than 1:10 to 1:320, whereas the antibody titer to HHV-6 remained less than 1:10. Another child with exanthem subitum complicated by acute hemiplegia had serologic evidence of primary HHV-7 infection. These two cases demonstrate a new relationship between HHV-7 and central nervous system symptoms.

Combination therapy with transfer factor and high dose stronger neo-minophagen C in chronic hepatitis B in children (HBe Ag positive).

This study mainly describes the efficacy of the combination therapy with Transfer Factor (TF) and high dose Stronger Neo-Minophagen C (SNMC) for HBV carrier children with HBe Ag positive chronic hepatitis. There were 12 patients, 10 males and 2 females aged from 7 months to 14 years 8 months. Liver biopsy was done in 11 patients, and the histopathological findings of the liver were chronic active hepatitis (8 cases) and chronic inactive hepatitis (3 cases). In 6 of 8 patients, HBe-Ag became negative (75%) within 18 weeks (mean 8 weeks) after the initiation of the combination therapy with TF and SNMC (HBe-seronegative), and 4 of these 8 patients (50%) became anti-HBe positive within 29 weeks (mean 15 weeks) (HBe-seroconversion). These results suggest that combination therapy with TF and high dose SNMC may be beneficial in the treatment of chronic hepatitis B in children.

Live varicella vaccine: prevention of nosocomial infection and protection of high risk infants from varicella infection.

For prevention of nosocomial infection, 25 infants including high risk patients received an emergency injection of live varicella vaccine. Three patients developed a rash within 5 days after vaccination, but their symptoms were mild. The other 22 showed no clinical symptoms and gave an immune response. Twenty-two patients receiving immunosuppressive therapy were vaccinated and 20 of them showed a positive response in the varicella skin test. Of 14 vaccinated patients with malignancies, 2 giving a positive skin test, later showed clinical varicella, but their symptoms were not severe. One case with ALL was immunized safely under very poor conditions during the first induction therapy. No complications were observed in any patients.