Toshiyuki Katoh

PCR on cerebrospinal fluid to show influenza-associated acute encephalopathy or encephalitis

BACKGROUND Except for Reyes syndrome, influenza-associated acute encephalopathy or encephalitis is not universally recognised. We did a multicentre study of laboratory and clinical data for patients with influenza-associated acute encephalopathy or encephalitis. METHODS In Nagoya, Japan, ten patients with acute encephalopathy or encephalitis associated with influenza-like illness were admitted to our hospitals between April, 1996, and March, 1997. We collected clinical, laboratory and serological data and assessed cerebrospinal fluid samples by PCR for influenza A and B. FINDINGS Seven patients, aged 22 months to 4 years, had evidence of recent influenza infection, six with type-A/Hong Kong (H3N2) and one with type B. The first sign in the central nervous system appeared within 2 days of fever in all but one patient. The first sign of involvement of the central nervous system was generalised convulsions in all patients. Two patients died, one had sequelae, and four survived without sequelae. PCR for influenza type A was positive for five patients. INTERPRETATION The results of PCR suggest that at least part of the influenza type A genome existed in the central nervous system. Influenza-associated acute encephalopathy or encephalitis in young children deserves wider recognition.

Severe cerebellar atrophy following acute cerebellitis

The clinical course and serial magnetic resonance imaging findings of a 4-year-old girl with acute cerebellar ataxia due to acute cerebellitis are described. Multifocal white matter lesions visualized by magnetic resonance imaging in both cerebellar hemispheres in the acute phase disappeared in the convalescent phase. Cerebellar signs became less prominent within 2 weeks, but mild ataxic gait remained. During the follow-up period of 32 months, there was a gradual development of cerebellar atrophy along with a recurrence of cerebellar ataxia.

Antley-Bixler syndrome in a sister and brother

SummaryA sister and brother both with the clinical and radiographic features of Antley-Bixler syndrome are reported. Their parents were first cousins. Analysis of these siblings and seven other patients reported in the literature indicates autosomal recessive inheritance for the syndrome.

Coronary risks after high-dose γ-globulin in children with Kawasaki disease

Abstract Objectives: The goals of the present study were to develop a predictive coronary risk scoring system after intravenous γ‐globulin (IVGG) therapy of any dose for the different preparations currently used in the treatment of children with Kawasaki disease and to determine the predictive value of the system. The previously reported scoring systems were based on treatment with high‐dose IVGG therapy at limited doses and were determined using investigative methods.

Successful pregnancy after coronary artery bypass grafting for Kawasaki disease.

Abstract A 13‐year‐old girl with a history of Kawasaki disease underwent coronary artery bypass grafting because of angina pectoris due to a giant coronary artery aneurysm on the left main trunk artery. Nine years after the operation, the patient had an uneventful pregnancy followed by a normal vaginal delivery. This is the first case of a successful pregnancy after coronary artery bypass grafting for Kawasaki coronary artery disease.

Automated determination of orotic acid, uracil and pseudouridine in urine by high-performance liquid chromatography with column switching

A column-switching high-performance liquid chromatographic method, requiring no sample preparation apart from filtration, is described for quantification of urinary orotic acid, uracil and pseudouridine. The analyses were carried out using a reversed-phase octadecylsilane-bonded column for sample clean-up and a cation-exchange column for separation; 5-20 microliters samples of urine were directly analysed, and more than 100 samples could be analysed consecutively. Each sample required only 30 min. Detection limits of these compounds were 5 pmol. Creatinine-related urinary uracil excretion was lowest in the newborn period (17.3 +/- 14.4 mumol/g of creatinine). A patient with partial ornithine transcarbamylase deficiency and his mother usually excreted a high level of uracil during the period of normal orotic acid excretion and normal serum ammonia level.

Automated Quantitative Analysis for Orotidine and Uridine/Thymine in Urine by High-Performance Liquid Chromatography with Column Switching

The measurement of urinary orotidine, uridine and thymine which are intermediates in pyrimidine biosynthesis, is important for screening and diag-nosis inborn ergors of metabolism, such as urea cyclel and pyrimidine metabo-lism disorders 2 , as well as the measurement of urinary orotic acid and uracil l,3. Several high-performance liquid chromatography (HPLC) methods have been reported by a number of researchers. However, these HPLC methods for uridine and thymine analysis require rather complicated sample preparation in order to obtain accurate results 1. Although, a simple method for measuring orotidine and orotic acid were recently reported 4, it is not capable of evaluating near-normal levels of orotidine.

Reference Values of Orotic Acid, Uracil and Pseudouridine in Urine

Measuring urinary orotic acid and uracil which are intermediates in pyrimidine biosynthesis, is important for screening inborn errors of metabolism, such as urea cycle[1] and pyrimidine metabolism disorders[2,3]. Pseudouridine is a degradation substance from transfer RNA and its measurement might be useful as a marker for cancer and cancer therapy evaluation[4]. Until now, there have been no reports of reference values classified by age. We are presenting the orotic acid, uracil and pseudouridine reference values measured by using high performance liquid chromatography (HPLC) with an automated column switching system[5].

Prevention of Coronary Artery Involvement in Kawasaki Disease by Early Intravenous High-Dose Gamma Globulin

Involvement of coronary arteries resulting from Kawasaki disease carries a risk of sudden death or ischemic heart disease. The purpose of this study was designed to select the high-risk patients, and then to evaluate the effect of high-dose intravenous (IV) intact immunoglobulin therapy in Kawasaki disease for the prevention of coronary artery involvement.

NEONATAL TYPE OF ARGININOSUCCINATE SYNTHETASE DEFICIENCY

Two autopsy cases of neonatal argininosuccinate synthetase (ASS) deficiency demonstrating the particular histological changes of the liver are presented.