bluuebunny/crossref_metadata_2025 huggingface dataset:
Plasma Concentrations of Atrial Natriuretic Peptide in Children with Persistent Hypertension
Authors: Ito Yuhei , Kinoshita Shouhei , Kato Hirohisa , Marumo Fumiaki , Ando Kenji
Published: October 2007

Plasma concentrations of human atrial natriuretic peptide (hANP) were determined in children with persistent hypertension and in age‐matched normotensive controls. We studied 40 children 6–7 years of age (Group A), and 74 children 13–14 years of age (Group B). There was no significant difference in plasma hANP concentration between hypertensives and normotensives in group A. In group B, the plasma hANP concentration in hypertensives (86.5 ±44.9 pg/ml: mean±SD) was significantly higher than in normotensives (58.8±40.4 pg/ml) (p <0.01). In group B, 24‐hour urinary excretion of sodium and fractional excretion of sodium were not significantly different between hypertensives and normotensives. The plasma hANP correlated significantly with the fractional excretion of sodium (p<0.05)and the 24‐hour urinary excretion of sodium (p<0.01) in normotensives. No such correlation was evident in the case of the hypertensives. The pathogenesis of increased plasma hANP concentration in older children with persistent hypertension remains to be investigated. In regard to the effects of hANP on renal function, hANP may contribute to the regulation of sodium handling in normotensive adolescents, whereas other regulatory mechanisms need to be considered in the case of hypertensives.
Myelopathy, Ascites and Pleural Effusion in Systemic Lupus Erythematosus
Authors: Waga Shinobu , Nukii Kiyotaka , Sugimoto Kazuhiko , Tanaka Hiroshi , Furukawa Hidetsugu , Sashinami Tsukasa
Published: October 2007

A 12‐year‐old girl with transverse myelopathy, massive pleural effusion and ascites as the presenting features of systemic lupus erythematosus (SLE), is described. All these features developed within a week, but other manifestations of SLE such as malar rash and arthritis were not seen during the illness. Investigations revealed positive direct Coombs' test, high titer of antinuclear antibodies, elevation of serum anti‐DNA antibodies, depressed complement activity, thrombocytopenia and proteinuria, indicating that she had SLE. A week after starting treatment with prednisolone 35 mg/day, the thrombocytopenia improved, and following an increase in dosage to 60 mg/day the pleural effusion and ascites diminished after two weeks, and the serological abnormalities improved after two months. The neurological disturbances were unchanged in spite of injections of methylprednisolone 1 g/day for three days from the 89th day of her illness. Review of other cases reported to be improved neurologically, suggested that the earlier initiation of corticosteroids in higher dosage might be beneficial in transverse myelopathy m SLE.
Serum Anti‐PT and Anti‐FHA Antibody Levels, and Agglutinin Titers after Administration of Accellular Pertussis Vaccines
Authors: Kuno‐Sakai Harumi , Kimura Mikio , Sato Yuji , Tsunoda Akira , Urano Takashi , Isomura Shin , Kamiya Hitoshi
Published: October 2007

Simultaneous evaluation of acellular pertussis vaccines from three manufacturers (Takeda, Biken, and Chiba) was performed. After receiving two doses of acellular pertussis vaccine in the form of DPT (diphtheria pertussis tetanus), both infants and children showed high serum anti‐PT (pertussis toxin) and anti‐FHA (fdamentous hemagglutinin) antibody levels. These levels were equivalent to those observed in children in the convalescent stage of natural pertussis infection. Children who received 2 doses of Biken vaccine showed higher anti‐PT and anti‐FHA antibody levels than those who received Takeda or Chiba vaccine. Elevation of agglutinin titers was observed in children who received either Takeda or Chiba vaccine. (Acta Paediatr Jpn 1989; 31: 120–126)
Breast‐Feeding and Fertility in Macau
Authors: da Silva Pinho , Ribeiro Isabel , Barbosa Maciel , Vieira Maria Helena
Published: October 2007

That fact that only a small percentage of women in Macau breast‐feed induced the need to investigate this attitude. In collaboration with the Maternal & Child Health Division of the W.H.O., Geneva, it was decided that a methodology which had already been applied in other places should be used to investigate the relation between breast‐feeding and fertility. In this way, information was obtained about the situation, within a given period of time which permitted a description of the duration of amenorrhea due to breast‐feeding, as well as the patterns of frequency and duration of breast‐feeding.
The Significance of Breast Milk Biotinidase
Authors: Oizumi Jun , Hirano Masahiko , Hayakawa Kou , Daimatsu Takaki , Zaima Koji
Published: October 2007

Biotinidase (EC 3.5.1.12) activity in human breast milk was first demonstrated by us, using a newly developed method. The enzyme activity was relatively low compared with that in human serum. However, its activity increased significantly with thiol‐reactive agents. Taurine and glutathione also enhanced the enzyme activity considerably. From these results, we speculate that biotinidase in milk would be protected from various inactivation‐mechanisms in the same manner as milk lipase. Biotinidase might play an important role in the intestinal absorption of the indispensable vitamin, biotin, during infancy, when enzymes in the pancreatic juice are still immature.
Clinical and Electroencephalographic Studies of Postencephalitic Epilepsy
Authors: Yoshioka Mieko , Kuroki Shigekazu , Mizue Hideshige
Published: October 2007

We retrospectively reviewed the clinical features and results of investigation of 62 patients with acute encephalitis or encephalopathy in the acute phase to identify the prognostic factors in the development of postencephalitic epilepsy. Patients who later developed epilepsy showed more marked disturbance of consciousness, convulsions, and seizure activities on EEG during the acute phase than those who did not. There was no relation between CSF abnormalities and postencephalitic epilepsy. Control of convulsions during the acute phase, especially of status epilepticus, seem to be important to improve the prognosis.
Neonatal Intracerebral Teratoma
Authors: Ishikawa Toshimi , Yasuhara Akihiro , Kobayashi Yohnosuke
Published: October 2007

A neonate, who presented with a large head but no neurological abnormalities, was diagnosed by ultrasound and CT scan as having a brain tumor. The pathological diagnosis was teratoma with matured tissues. The literature is briefly reviewed and the clinical manifestations and histological classification are documented.
Chronic Epstein‐Barr Virus Infections Associated with Coronary Aneurysms
Authors: Kobayashi Ichiro , Hamada Isamu , Tomizawa Kazuhiro , Kikuta Hideaki , Sakiyama Yukio , Matsumoto Shuzo
Published: October 2007

We recently reported virological studies in a patient with chronic active Epstein‐Barr virus (EBV) infection in which T‐lymphocytes were infected, and coronary aneurysms were detected by echocardiography. We report here the clinical features of this patient and suggest that EBV may cause coronary aneurysms and that an echocardiographic study should be performed in patients with chronic active EBV infection.
Natural Killing of Varicella‐Zoster Virus (VZV)‐Infected Fibroblasts in Normal Children, Children with VZV Infections, and Children with Hodgkin's Disease
Authors: Ihara Toshiaki , Kamiya Hitoshi , Starr Stuart E. , Arbeter Allan M. , Lange Bevery
Published: October 2007

We studied mononuclear cell (MNC)—mediated natural killing (NK) of varicella‐zoster virus (VZV)—infected fibroblasts in normal children, children with VZV infections, and children with Hodgkin's disease. NK activity was tested in 18hr 51Cr release assays. NK activity for adults was significantly higher than that for children 1–3 years old or 4–6 years old (p< 0.05). Serological status did not affect NK activity. NK activity in normal children was not increased 4–6 weeks after immunization with varicella vaccine. Seven normal children with natural varicella showed significantly higher NK activity against VZV‐infected and unin‐fected targets. Eight immunosuppressed children with herpes zoster showed significantly reduced NK activity within 72 hours of the onset of herpes zoster. However, their NK activity rose to the normal level one to two weeks later. Children with Hodgkin's disease had low NK activity. These results suggested that NK cells might play an initial defensive role in VZV infections, and that low NK activity in immunocompromised hosts might contribute to their high incidence of herpes zoster.
3‐Year Study for the Prevention of Perinatal HBV Infection Under the Standard Method of the Ministry of Health and Welfare, Japan
Authors: Kawabe Yoshikazu , Sugiyama Kohachiro , Wada Yoshiro , Yamada Katsumi
Published: October 2007

We present the first report on a 3‐year study for the prevention of HB virus infection under the standard method of the Ministry of Health and Welfare, Japan. We observed 50 children for three years, and obtained the following results: 1) Many children (57%) born to HBeAg‐positive mothers maintained HBs‐antibody titers higher than 10, expressed by the cut‐off index based on the RIA system; 2) No case became an HB virus carrier after one year; 3) Six children born to Hkantibody positive mothers had HBs‐antibody titers below 5. Children born to HBe‐antigen positive mothers were mostly affected by the HB virus, so that their HBs‐antibody titers were maintained at high levels. In contrast, HBs‐antibody titers of children born to HBe‐antibody positive mothers decreased, probably because of lack of exposure to the HB virus.
Effects of Booster HB Vaccine in Preventing Hepatitis B Virus Infection from Mother to Child
Authors: Kunitorni Taiji , Ikeda Masanori , Oda Megurni , Yarnada Gotaro , Eguchi Katsuto , Ubuka Keiko , Morita Hideo , Kurashige Takanobu
Published: October 2007

Hepatitis B immune serum 200 IU was injected intramuscularly to 127 infants born to hepatitis B e antigen positive mothers immediately after birth and at two months of age, and if necessary at four months. HB adjuvant vaccine 20 μg was injected subcutaneously at three, four, and six months. If antibodies to hepatitis B surface antigen were 22 or less, one to four doses of booster vaccine 20 μg were given. The first booster vaccination was given in 8.7% of cases by the age of one year, in 19.3% by 18 months, in 23.9% by 24 months, and in 36.2% by 30 months; thus 2‐to‐3‐year‐old infants were more frequently vaccinated than younger ones. This implies that 80%‐90% of infants could be protected from vertical transmission of hepatitis B virus (HBV) up to the age of three years, if a booster vaccine was given to those born to HBeAg positive mothers not later than 24 months. Infants obtained enough antibody to prevent HBV infection by receiving one booster vaccination in 34 cases, by two in nine, by three in five, and by four in two.
Methylsuccinate and Mesaconate in Urine of Patients Treated with Sodium Benzoate
Authors: Maeda Eisaku , Matsuo Masafurni , Saiki Kayoko , Nakarnura Hajirne , Matsuo Tarnotsu , Takernine Hisao
Published: October 2007

Two unusual peaks were found on gas chromatography of urine from four hyperammonemic patients treated with sodium benzoate. These peaks were identified by gas chromatography/ mas spectrometric analyses as methylsuccinate and mesaconate. Of the two unusual substances, methylsuccinate was found to be a contaminant of sodium benzoate administered for the treatment of hyperammonemia. However, mesaconate was not a contaminant of sodium benzoate, though it could be detected in all urine samples from hyperammonemic patients treated with sodium benzoate. Mesaconate can be produced from methylsuccmate in viva Considering that mesaconate is an inhibitor of fumarase, the toxic effects of sodium benzoate may be attributable to the mesaconate. It is recommended that methylsuccinate‐free sodium benzoate should be used for the treatment of hyperammonemia.
The Effect of Reconstituted Bovine Surfactant on Pulmonary Mechanics in Infants with Respiratory Distress Syndrome
Authors: Tamura Masanori , Kawano Toshio
Published: October 2007

We performed sequential measurements of pulmonary mechanics following instillation of reconstituted bovine surfactant (surfactant TA) in 10 neonates with severe respiratory distress syndrome. The respiratory compliance did not increase significantly until six hours after surfactant administration, while the arterial oxygenation and radiographic appearance improved rapidly after surfactant therapy. Six infants developed clinically significant PDA between 24 hours and 48 hours after surfactant therapy, when the compliance decreased temporarily after initial improvement until the PDA was closed pharmacologically. In six cases airway resistance rose transiently following surfactant treatment, whereas PCO2 fell significantly in all cases by six hours after surfactant administration. We recommend that only inspired oxygen concentration should be adjusted without changing the pressure setting of a respirator for at least three hours after surfactant therapy.
Effect of Long‐Term Treatment with Massive Doses of 1α‐Hydroxyvitamin D3 on Calcium‐Phosphate Balance in Patients with Vitamin D‐Dependent Rickets Type II
Authors: Takeda Eiji , Yokota Ichiro , Saijo Takahiko , Kawakami Ikuo , Ito Michinori , Kuroda Yasuhiro
Published: October 2007

Three patients with vitamin D‐dependent rickets type II were given massive doses of 1α‐hydroxyvitamin D3 for 29 to 36 months and their calcium‐phosphate balance was studied during treatment and one month after cesation of treatment. During treatment fasting hypercalciuria was observed in patient 1 and an increased rate of calcium excretion after calcium loading in patients 1 and 2. In these patients, calcium excretion was parallel with the serum 24, 25‐dihydroxyvitamin D. These findings suggested that the responsiveness to 1,25‐dihydroxyvitamin D improved during long‐term treatment of these two patients with vitamin D‐dependent rickets type II.
Renal Osteodystrophy in Patients on Continuous Ambulatory Peritoneal Dialysis
Authors: Hisano Satoshi , Yamane Ichiroh , Ueda Kohji , Kawagoe Masataka
Published: October 2007

Twelve patients on continuous ambulatory peritoneal dialysis (CAPD) were studied in order to evaluate the progression of renal osteodystrophy (ROD). All patients received doses of 0.01 ‐ 0.02 μg/kg of 1 alpha vitamin D3(1α ‐ D3) and 0.1 ‐ 0.15 g/kg of calcium carbonate for 12 ‐ 18 months. Serum total protein, total calcium (Ca), creatinine, inorganic phosphate alkaline phosphatase (ALP), and n‐terminal parathyroid hormone were measured regularly. The radiological bone appearance for ROD or rickets and the height standard deviation score were compared between the outset and the end of this study. An increase of Ca values and a decrease of ALP values correlated with a suppression of hyperparathyroidism, and the hyperphosphatemia was controlled in the majority of our patients throughout this study. Two patients had ROD and rickets at the outset of this study, and all patients but one had neither ROD nor rickets at the end of this study. Growth retardation improved or remained stable in 10 patients. Our results indicate that adequate doses of 1α ‐ D3 and calcium carbonate are effective in the prevention of ROD and rickets in patients on CAPD.
Organic Acid and Acylcarnitine Profiles of Glutaric Aciduria Type I
Authors: Matsumoto Masahiro , Matsumoto Isamu , Shinka Toshihiro , Kuhara Tomiko , Imamura Hiroaki , Shimao Satoshi , Okada Toshio
Published: October 2007

Urinary organic acid and acylcamitine profiles from a 2‐month‐old boy were studied by gas chromatography‐mass spectrometry and fast atom bombardment mass spectrometry. The patient excreted large amounts of glutaric acid and significant amounts of 3‐hydroxyglutaric acid, glutaconic acid and glutarylcarnitine, and his serum glutaric acid level was markedly elevated. Thus he was chemically diagnosed as having glutaric aciduria type I (GAI). In addition to the above metabolities previously described in GAI, significantly increased excretion of 2‐ketoglutaric acid, succinic acid, adipic acid, adipylcarnitine, suberic acid and azelaic acid was found. 2‐Ketoadipic acid methylsuccinic acid and ethylmalonic acid were also detectable, suberylcarnitine was not increased, and dehydroadipylcarnitine was decreased in his urine. These results suggest that excess glutaryl‐CoA causes the competitive inhibition of the dehydrogenation of adipylCoA to dehydroadipyl‐CoA and results in an increase of adipic acid and adipylcarnitine and a decrease of dehydroadipylcamitine. It is also suggested that oxidative decarboxylation of 2‐ketoglutaric acid to succinyl‐CoA is inhibited by high levels of glutaryl‐CoA, and that the dehydrogenation of succinic acid to fumaric acid is inhibited owing to the increased glutaric acid derived from excess glutaryl‐CoA. These results indicate that gas chromatography‐mass spectrometry is the most appropriate and accurate method for the differential chemical diagnosis of GAI and glutaric aciduria type II.
Terminal Deletion of Chromosome 10q and Its Clinical Features
Authors: Kagasaka Ryoichi , Morohoshi Teruaki , Sawada Yohko , Fujiwara Masaki
Published: October 2007

A male case with terminal deletion of chromosome 1Oq has growth retardation, craniofacial dysmorphism, congenital heart disease and other minor anomalies. The karyotypic formula is 46, XY, del (lo), (q26.1→q ter) by a high resolution G‐banding staining. There are few differences in clinical features between our case and the previously reported cases.
Effect of Continuous LDL Apheresis with Dextran‐Sulfate Cellulose Column System on a Child with Homozygous Familial Hypercholesterolemia
Authors: Yoshimoto Masaaki , Tsuru Akira , Sawai Tomoko , Kinoshita Ei‐ichi , Baba Tsuneyoshi , Yokoo Tetsuya , Yanagi Tadamichi , Tsuji Yoshiro
Published: October 2007

A new system for selective low density lipoprotein apheresis with an automated regenerating column using dextran‐sulfate (DS) as ligand was evaluated for six months in a 13‐year‐old boy homozygous for familial hypercholesterolemia. Two columns each containing 150 ml of DS cellulose were alternately used after rinsing with a regenerating solution. The patient could well tolerate the volume in the system. The values of plasma total cholesterol decreased by 79.4 ± 4.9% of the pretreatment levels after a total of 5ℓ plasma apheresis, while those of high density lipoprotein cholesterol did not change. Although the values of CH50 decreased, no adverse reaction was seen during the period of treatment. It was concluded that the present apheresis system was highly efficacious and safe for children homozygous for the mutant LDL receptor gene.
Childhood Asthma in Thailand
Authors: Tuchinda Montri
Published: October 2007

Asthma is common in Thai children with a prevalence of about 4%. A prospective study of 2,000 Thai asthmatic children showed boys to be more frequently affected than girls, and fifty percent of patients experienced their first attack within the first 2 years of life. Attacks usually occurred in winter and the rainy season. 63.15% of patients had asthma associated with other allergic diseases and 79.25% had a family history of allergic diseases. Positive skin tests to important antigens were noted in 93.7% of cases, and house dust was the most common. Pharmacokinetic studies of theophylline were made in Thai asthmatic children after oral administration revealed that the elimination half‐life of theophylline was longer than that observed in subjects in Western countries. Long‐term follow up of 247 Thai asthmatic children revealed that in 37.2% of patients, symptoms ceased at the age of 15 years. The factors which indicated a favorable prognosis included male sex, mild or moderate illness, negative skin tests or positive skin tests to only a few allergens.
Changes of Left Ventricular Isovolumic Relazation Time with Growth in Children
Authors: Hayashi Yurika , Watanabe Koji , Kishida Kenji , Haneda Noriyuki , Iwatani Hajime , Nishio Toshikazu , Mori Chuzo
Published: October 2007

We investigated the changes of left ventricular isovolumic relazation time (LVIRT) with growth and the factors influencing them during childhood, in a 10‐year follow‐up stury.We measured the body height(BH), body weight (BW), lean body weight (LBW), heart rate (HR), Blood pressure (BP), left ventricular muscle volume index (LVMVI), and LVIRT corrected by the preceding R‐R interval) IRT/R‐R), of 187 healthy children at 3‐year intervals (6,9,12,15 years old). IRT/R‐R were prolonged with growth in boys and girls. At the ages of 12 and 15, the IRT/R‐R of girls were longer than those of boys. Significant correlation coefficients for IRT/R‐R on systolic and diastolic, BW and LVMVI were 0.28, 0.31, 0.20, 0.28, respectively. These data suggest that(1)IRT/R‐R is mainly determined by the diastolic BP,(2) left ventricular diastolic function of children develops with growth of body size, especially BW, and(3) left ventricular early diastolic function in adolescents isrelated to sexual maturation.
Effects of Intravenous Nitroglycerin on Hemodynamics in Neonates with Refractory Congestive Heart Failure of PFC
Authors: Tamura Masanori , Kawano Toshio
Published: October 2007

(1) Continuous intravenous infusion (2–10 μ/kg/min) of nitroglycerin (TNG) was administered to 20 neonates consisting of 17 with refractory congestive heart failure and 3 with PFC (Persistent Fetal Circulation). (2) AT doses of 2–4 μ/kg/min, there were no significant changes in heart rate or systemic blood pressue. (3) At doses of 2–4 μ/kg/min, the CVP was significantly reduced and urnary output increased. (4) Echocardiograms revealed a significant decrease in LVS at 4 – 5 μ/kg/min. In addition, the EF and cardiac output were significantly increased at 2 – 5 μ/kg/min. (5) The right ventricle systolic time interval (STI) was reduced significantly at 2 μ/kg/min. The left ventricle STI was not reduced below doses of 4 μ/kg/min. (6) In the dose range 2 – 5 μ/kg/min, TNG is an effective and safe vasodilator in the treatment of refractory congestive heart failure or PFC in neonates.
Ectopic Nesidioblastosis
Authors: Seki Syuichiro , Ikenoue Tsuyomu , Murakami Naoki , Hokanishi Hisahiko , Kikuura Toyofumi , Tokunaga Masayoshi , Akiyama Hiroshi , Hayakawa Kunio
Published: October 2007

We observed one case of ectopic nesidioblastosis. A solitary nesidioblastoma was observed in the interior wall of the duodenum. Ectopic nesidioblastosi has not been previously reported, but our experience suggests the necessity of examination for ectopic pancreas in cases of SIDS or its near‐miss.
Cerebral and Abdominal Arterial Hemodynamics in Preterm Infants with Patent Ductus Arteriosus
Authors: Ichihashi Kou , Shiraishi Hirohiko , Endou Hideki , Kuramatsu Toshihiro , Yano Sadayuki , Yanagisawa Masayoshi
Published: October 2007

Using Doppler echocardiography we evaluated the effect of ductal shunt flow on the cerebral and abdominal arterial blood flow in 25 preterm infants. Eligible for inclusion in this study were healthy preterm newborn infants. They were divided into two groups based on their gestational age: group A, 33‐36 weeks (15 infants) and group B, 28–32 weeks (10 infants). Two‐dimensional Doppler echocardiograms were obtained in each infant during the first 8 hours of life and repeated every 6–12 hours until no ductal shunt flow could be detected. Flow in the ductus arteriosus, the basilar artery and the coeliac artery were examined. Closure of the ductus arteriosus occurred significantly later(p< 0.05) in group B than in group A. Pulsatility indices of flow in the basilar and coeliac arteries were high when the ductus was patent, decreasing to a fixed level with closure. This study suggests that a shunt of the patent ductus arteriosus (PDA) adversely influences the cerebral and abdominal blood flow in preterm infants.
Analysis of Breathing Pattern in Children with Asthma
Authors: Tanaka Tetsuro , Shimazaki Tetsuya , Kusakawa Isao , Ogihara Masaaki , Honda Teruo , Katayama Masao , Miyasaka Katsuyuki
Published: October 2007

The breathing pattern in children with asthma was studied, using respiratory inductive plethysmography. The subjects were 31 children with mild to moderate asthma (15 males, 16 females; aged 6∼15 years, average 11 years). The respiratory rate was 25.5±10.7/min. (mean ± S.D.) during asthma attacks and 18.4 ± 5.0/min. between attacks with a significant increase during attacks (P < 0.001). The expiratory time was 1.774 ± 0.833 second during attacks, and 2.125 ± 0.602 seconds between attacks. The expiratory time to total respiratory time ratio (TE/TTOT) was 0.631 ± 0.056 during attacks and 0.608 ± 0.035 between attacks. Thus there was a slight prolongation at the time of attack, but the difference was not significant. The tidal volume was increased slightly during mild and moderate attacks. VT/TI (mean inspiratory flow) was increased during attacks. The respiratory pattern was thoracic during attacks and changed to the abdominal pattern with improvement.
Non‐Icteric Fulminant Hepatitis and Reye's Syndrome: Comparison of Laboratory Data
Authors: Shibao Kyoko
Published: October 2007

Twenty‐eight patients with suspected Reye's syndrome (RS) were seen in our Department from 1974 through 1987. Liver biopsy confirmed the diagnoses of RS and nonicteric fulminant hepatitis (NIFH) in 7 and 5 cases, respectively. NIFH was the most common RS mimicker. Total bilirubin, LDH and serum ammonia levels showed no significant differences between RS and NIFH. However, the levels of serum GOT and GPT were significantly higher in the NIFH group. Serum and urinary carnitine levels were measured in both groups, but the results were inconclusive. Amino acid analysis in one RS and two NIFH patients showed no significant differences in the ratio of branched chain to aromatic amino acids. However, one RS patient showed a high level of lysine. Histological findings in the liver of two NIFH patients showed minor mitochondria1 swelling and microvesicular fat, but the major finding was hepatic necrosis. Our experience indicates that NIFH and RS cannot be differentiated by routine laboratory tests. Liver biopsy is essential for the accurate diagnosis of RS.
Mitochondrial Changes and Carnitine Status in Fasting Rats
Authors: Matsuyuki Makado
Published: October 2007

To elucidate mitochondrial morphology and related pathophysiology in the fasting state, liver mitochondrial area, plasma levels of carnitine and non‐esterified fatty acids were measured in fed (N = 30) and fasted for 5 days (N = 28) rats. The rats were divided into three groups according to body weight: 100g‐group (aged 5 weeks), 200g‐group (aged 7–8 weeks), 400g‐group (aged 15–16 weeks). Electron microscopy of the liver revealed mitochondrial enlargement in fasted rats. There were significant differences in mitochondrial area measured by digitizer, between fasted groups with 100g and 200g body weights and the compatible fed groups. The grade of mitochondrial swelling was inversely proportional to the body weight (i.e. the age). The fasting induced decreased plasma levels of free carnitine and increased plasma levels of acylcarnitine. Plasma free carnitine levels in all fed rats appeared to be directly correlated to the body weight. The above mitochondrial swelling and abnormal carnitine status are hallmarks of Reye's syndrome (RS). These changes are not specific to RS but might be attributable to fasting.
Carnitine Metabolism in Rats with 4‐Pentenoic Acid Induced Fatty Liver
Authors: Yuge Ken
Published: October 2007

The effects of 4‐pentenoic acid, a chemical claimed to induce fatty liver morphologically similar to that seen in Reye's syndrome, on carnitine metabolism and on hepatic histology were studied. Male Wistar rats were injected with 50 mg/kg doses of 4‐pentenoic acid intraperitoneally every four hours over a period of 82 hours. Control rats received a similar volume of saline instead. The animals were then sacrificed at 82 hours. Liver, serum and urine were collected and stored in dry ice. The concentrations of free, acyl‐, and total (free plus acyl) carnitines determined in serum were found to be significantly decreased in the rats which had received 4‐pentenoic acid, while the concentrations of acyl‐ and total carnitines were significantly elevated in urine. In the liver tissue, the concentrations of short chain‐ and medium chain‐acylcarnitines tended to be elevated, but the differences were not statistically significant. These results suggest that enhanced acylation of free carnitine with metabolites of 4‐pentenoic acid, and excretion of the resulting acylcarnitine into urine are likely mechanism of the 4‐pentenoic acid‐induced hypocarnitinemia. Liver histology revealed marked fatty change with minute fat droplets similar to those observed in Reye's syndrome, and very slight alteration in mitochondrial configuration.
The Effects of Salicylate on Ketogenesis, Gluconeogenesis and Urea Production in Rat Liver Perfusion
Authors: Terada Naoto , Kizaki Zenro , Inoue Fumio , Kodo Naoki , Ochi Masaharu , Furukawa Nobuaki , Kinugasa Akihiko , Sawada Tadashi
Published: October 2007

In order to elucidate the relation between the hepatotoxicity of salicylate (SA) and the pathogenesis of Reye's syndrome (RS), urea production, gluconeogenesis and ketogenesis were investigated in isolated perfused rat livers in the presence of salicylate (SA) and oleate. Although urea formation from 0.5 mM NH4Cl, 2 mM ornithine and 0.3 mM oleate was not inhibited by infusion of SA, 3 mM SA caused a 26% decrease of ketogenesis, 85% decrease of 3‐hydroxybutyrate/acetoacetate ratio (30HB/AcAc) and 45% increase of oxygen consumption. Glucose production from 2 mM pyruvate in the presence of 0.3 mM oleate decreased by 33% after administration of 3 mM SA, and 30HB/AcAc ratio also decreased by 33%. The decrement of gluconeogenesis and that of the 30HB/AcAc ratio were very close.

These results suggested that ATP production was maintained but that the intra‐mitochondrial redox state was changed to a more oxidized state after SA administration in perfused rat livers. This change in redox state could be responsible for the decrease of gluconeogenesis.

Metabolic characteristics found in RS were not obtained by infusion of 3mM SA and 0.3 mM oleate in rat livers. Therefore, some other factors in addition to SA seem necessary to establish an animal model of RS.
Group C Rotavirus Infection and Infiltration
Authors: Ishimaru Yoshiro , Nakano Hiroko , Oseto Mitsuaki , Yamashita Yasutaka , Kobayashi Nobumichi , Urasawa Shozo
Published: October 2007

We recently found many cases of gastroenteritis involving older children and sometimes other members of their families. We suspected epidemiological infiltration of an atypical rotavirus (RV). PAGE analysis of 130 cases in 1985 and 1986 revealed that the RNA profiles were similar to that of group C RV in 12 cases. We performed IEM analysis on one of the strains and found it to be group C. The group C RV was detected for 2 months toward the end of the outbreak of group A RV infection. The ages of the group C RV infected patients were 4 years or older. We also studied the prevalence of antibody to group C in the stored sera that had been collected since 1971. Antibody was seldom found in the sera of patients under 3 years of age but it was found in 20–40% of those who were 4 years or older. The clinical manifestations of the group C RV infection were similar to those of group A, except that body temperature was slightly lower and other symptoms were generally milder.
Mid‐Cervical Malignant Pleomorphic Adenoma Prsenting With Upper Airway Obstruction.
Authors: Hashizume Kohei , Nakajo Toshio , Kawarazki Hideo , Iwanaka Tadashi , Kanamori Yutaka , Tanaka Kiyoshi , Utsuki Tadahito , Uno Kaoru , Mizuno Mashiro
Published: October 2007

The case of a 1 year and 6 months old girl with pleomorphic adenoma arising from the upper middle part of the neck is reported. Although the tumor invaded the hyoid bone, it was removed completely and symptoms of upper airway obstruction disappeared. We suspect it may have originated from thyroglossal duct structures.
Improved Outcome for Infants Weighing Less then 750 Grams at Birth: Effects of Advances in Perinatal Care, Infection Prevention and Maternal Transport for Fetus
Authors: Kukita Johji , Yamashita Hironori , Minami Taketsugu , Fujita Ichiro , Koyanagi Takashi , Ueda Kohji
Published: October 2007

Between 1980 and 1987, we investigated the outcome for 20 infants with birth weights <750 g, admitted to the neonatal unit of Kyushu University Hospital. All infants were delivered at the hospital. Seven infants (35%) survived and were discharged. Comparing the first and second 4 year periods, the survival rate improved from 17% (1 of 6 infants) to 43% (6 of 14 infants). Intensive perinatal care, prevention of infection and early transport of mothers of high‐risk babies improved the outcome. In the long‐term, among the 7 survivors, 1 had psychomotor retardation, 1 had epilepsy and the other 5 were normal neurologically. Growth in height and weight of these children remained below the ‐2SD levels until 2 years of age, thereafter they began to catch up. Growth in head circumference increased to a level above the ‐2SD value from 2 years of age. With intensive perinatal care, the outcome for infants weighing <750 g is improving, and good results may be expected.
Pseudo‐Chediak‐Higashi Anomaly in Acute Myeloid Leukemia (M2) of Childhood
Authors: Aonuma Kesashi , Komiyama Atsushi , Akabane Taro
Published: October 2007

The frequency and clinical significance of the pseudo‐Chediak‐Higashi (PCH) anomaly were studied in 20 children with acute myeloid leukemia (AML) M2 in the FAB nomenclature. PCH granules were recognized as giant eosinophilic granules, measuring up to 5μ, in the cytoplasm of leukemic cells on smears. At the electron microscope level, most PCH granules were round to oval and outlined by a limiting membrane, and contained homogeneous, granular, crystalloid, rod‐like or myelin‐like materials. The PCH anomaly was demonstrable in five (25.0%) of the 20 patients, which indicates that the anomaly is not rare in childhood AML M2. There were no differences between PCH anomaly‐positive and PCH anomaly negative groups with regard to hepatosplenomegaly, hemoglobin levels, white blood cell counts, bone marrow cellularity, t(8q‐, 21q+) chromosome abnormalities or prognoses. Circulating leukemic cells were observed less frequently in the PCH anomaly‐positive group than in the PCH anomaly‐negative group (p <0.05); the leukemic cells were not demonstrable in three of the five patients in the former group, although they were detected in all 15 patients in the latter group. The existence of PCH granules and/or a defect of the cytoskeleton responsible for the PCH anomaly in leukemic cells may impede their movement from the bone marrow to the peripheral blood.
Septicemia in Immunocompromised Children and Their Intestinal Flora
Authors: Yoshioka Hajime , Sakata Hiroshi , Fujita Kozo , Kakehashi Hitoshi , Oka Toshiaki , Kaeriyama Masato
Published: October 2007

Quantitative fecal bacteriology was performed in eight immunocompromised children with septicemia. The most marked change observed was suppression of the anaerobic bacteria. In seven patients, the predominant organisms were aerobic gram‐negative bacilli (GNB), and in six of these were the same as the causative organism of the septicemia. Thus, overgrowth of GNB in the gastrointestinal tract may result in invasion of the blood stream and septicemia in immunocompromised patients. To prevent this complication it is necessary to allow the normal intestinal flora to be maintained in these patients as long as possible. Antibiotics should therefore be prescribed with caution. For the same reason, use of immunosuppressive drugs should be kept to a minimum. Bacteriological examination of the stool and pharynx is useful in the management of immunocompromised patients.
Mass Screening for Neuroblastoma in Japan
Authors: Sawada Tadashi , Nagahara Noboru , Yamamoto Keiko , Okabe Ikuo , Hanawa Yoshiyuki , Hashizume Kouhei , Matsui Ichiro , Okawa Haruo , Tsunoda Akio , Sakurai Minoru , Ueda Kazuhiro , Kimura Shigeru , Nakagawa Akira , Hata Yoshinobu , Takeda Takeo , Ohi Ryoji
Published: October 2007

The present status of NB mass screening in Japan, which is the first national trial in the world, is presented. This program was conducted in cooperation with infants' mothers, local health centers, screening centers and hospitals. Three hundred and thirty‐seven cases detected by the program, from the start in Kyoto in 1974 to the end of 1989, are analyzed. As many cases were detected at early stages, 97% of them were expected to be cured. Several clinical, technical and programmatic problems are also discussed.
Antibody Response Determined with Antibody‐Dependent Cell‐Mediated Cytotoxicity (ADCC), Neutralizing Antibody, and Varicella Skin Test in Children with Natural Varicella and After Varicella Immunization
Authors: Ihara Toshiaki , Kato Takashi , Torigoe Sadayoshi , Oitani Kaichi , Isaji Machiko , Ito Masahiro , Kamiya Hitoshi , Sakura Minoru
Published: October 2007

Humoral and cellular immune responses were examined in 115 children with varicella and in 21 children who had been immunized with live varicella vaccine. Antibody determined with antibody‐dependent cell‐mediated cytotoxicity (ADCC) was detected earlier than neutralizing antibody (NT) in both groups. While humoral and cellular immunity were detected simultaneously in the natural varicella group, cellular immunity was detected earlier than humoral immunity in the immunized group. This suggests that ADCC, rather than NT, is involved in the early stages of recovery from varicella‐zoster virus (VZV) infection.
Benign Familial Neonatal Convulsions: Clinical Features of the Propositus and Comparison with the Previously Reported Cases
Authors: Wakai Shuji , Tachi Nobutada , Chiba Shunzo , Ishikawa Yukitoshi , Okabe Minoru , Minami Ryoji , Kibayashi Masahiro
Published: October 2007

A family with benign familial neonatal convulsions (BFNC) was presented. The propositus had his first episodes of cyanosis on the second day after birth. Thereafter, he also experienced multifocal clonic and/or focal clonic seizures. Between the seizures he appeared well and was essentially normal upon physical examination. Treatment with phenobarbital (4 mg/kg/day, p.o.) was started, and subsequently, he had no further seizures until 3 months of age. At the age of 4 months, he was again admitted to the hospital because of generalized tonic‐clonk seizures. The findings of ictal EEG at that time were characterized by fast spiking with increasing amplitude during the tonic phase. During the clonic phase, there were repetitive bursts of spikes or sharp waves mixed with persisting muscle potentials. The termination of the convulsion was characterized by general voltage depression. Reference to previously reported cases of BFNC revealed that 10–15% of patients with this disorder had epilepsies later in life.
Generation and Function of αδ T Cells after Allogeneic Bone Marrow Transplantation in Humans: Comparison in Absence or Presence of HLA‐Matched or Mismatched Thymus
Authors: Nagasawa Masayuki , Morio Tomohiro , Takagi Shuji , Yata Junichi
Published: October 2007

We have observed two patients who exhibited an exclusive increase of δ TCS1+ subset of αδ T cells in the peripheral blood after bone marrow transplantation (BMT). In one case with severe combined immunodeficiency (SCID) who received haploidentical BMT from his father, αδ T cells appeared only after thymus transplantation. However, his T cell‐mediated immunity remained severely defective despite the generation of T cells of donor origin. In the other case with aplastic anemia, δ T CS1‐αδ T cells began to increase in the peripheral blood later. This indicates that the thymus is necessary for the generation of αδ T cells and that the δ TCS1+ subset is dominant in the early stages of their ontogeny. δ TCS1+ T cell lines were established from both patients, and allo‐reactivity was investigated. The cell line from the latter case reacted to recipient cells in a mixed lymphocyte reaction, but did not show cytotoxity to the allogeneic cells including recipient cells. The other cell line, from the former case, did not react to either donor or recipient cells. This indicates that an intact thymus is needed for αδ T cells to acquire allo‐reactivity. Both cell lines showed MHC non‐restricted cytotoxity against NK‐sensitive target cells.
The Effect of Immunoglobulin on Immune Complexes in Patients with Kawasaki Disease (MCLS)
Authors: Koike Rintaro
Published: October 2007

In mucocutaneous lymphnode syndrome (MCLS) IgG and IgM class immune complex (IC) showed peak levels on day 10 to 20 after the onset of the disease respectively, and a transient decrease in IgM class IC was detected at the beginning of intravenous high dose γ‐globulin therapy (IHDGT). In in vitro examination, when γ‐globulin was added to the serum samples of MCLS patients, IgG class IC increased while IgM class IC decreased. Consumption of complements was also observed in these samples. Disposition of IC in the reticuloendothelial system seemed easier by both binding of γ‐globulin to IC and increasing complement bound to IC resulting in increasing complement‐binding site. These results suggest that IHDGT is effective for the treatment of IC in MCLS. Sodium dodecyl sulfate‐polyacrylamide gel electrophoresis (SDS‐PAGE) revealed 14 bands from 300 K.D. to 42 K.D. in IC of MCLS patients in the acute phase of the disease and prior to treatment. The immunoblotting method revealed that IC contains IgG, IgM, IgE, IgA, Clq, C3, and C4.
An Epidemic of Meningococcal Disease in Karachi (Pakistan): A Study of Children
Authors: Dure‐Samin Akram , Mubina Agboatwalla , Azra Yasmin
Published: October 2007

A prospective study of 112 cases of epidemic meningococcal infection in the paediatric population is presented. The natural course of the disease, its complications, prognostic factors and therapeutic agents were studied. The cases were graded according to fixed criteria of severity which correlated well with the outcome of the disease. The gram‐stain of the CSF was a sensitive and a quick method of diagnosis and crystalline penicillin a cost‐effective therapy giving a good response within 48 hours in 90% of the cases.
Massive Intestinal Albumin Loss after Fontan Operation
Authors: Sano Tetsuya , Tajiri Hitoshi , Nakajima Toru , Matsushita Tohru , Kayatani Futoshi , Kanaya‐lda Shinobu , Ozaki Yoshikazu , Nose Osamu , Okada Shintaro
Published: October 2007

Massive intestinal protein loss was demonstrated and the site of loss determined by abdominal scintigraphy with 99mTc‐labeled human serum albumin in a 9‐year‐old girl following the Fontan operation for pulmonary atresia with intact ventricular septum. Significant activity accumulating in the lower small intestine and moving with its contents into the colon were shown. Her condition may have resulted from intestinal lymphangiectasia, caused by increased central venous pressure. Abdominal scintigraphy with 99mTc‐human serum albumin is useful in the diagnosis of protein‐losing enteropathy.
Transcutaneous Bilirubinometry: Effect of Postnatal Age
Authors: Yamauchi Yoshitada , Yamanouchi Itsuro
Published: October 2007

The purpose of this study was to determine whether the postnatal age might affect the accuracy and reliability of transcutaneous bilirubin measurements. A total of 576 transcutaneous bilirubin measurements were performed on 336 Japanese full‐term breastfed newborn infants during the first 12 days of life. We divided them into three groups according to postnatal age and studied the effect of postnatal age on the accuracy and reliability of TcB measurements. Our study revealed that the most reliable site for transcutaneous bilirubin measurements changed from the forehead to the sternum with advancing postnatal age (i.e. forehead for0–3 days, chest for4–5 days, sternum for 6 days and later).
A Case of Citrullinemia with Abnormal Messenger RNA for Argininosuccinate Synthetase
Authors: Nukada Osamu , Uchiyama Chuzou , Ubuka Satoko , Yoda Tadao , Kobayashi Keiko , Ichiki Hitoshi , Saheki Takeyori
Published: October 2007

A male neonate, thus far healthy and fed with breast milk, developed rapidly increasing apathy on the third day of life. Sucking became poor and hyperhidrosis was present. Hyperammonemia (3,305 µg/dl) was noted. He became comatose and died on the fourth day. There was a profound derangement of amino acid concentration in the body fluids, with highly elevated citrulline levels (4.70 µmol/ml in serum and 8.47 µmol/ml in urine). Autopsy showed diffuse pulmonary bleeding, as the only noteworthy pathological finding. The liver contained no detectable argininosuccinate synthetase (ASS) activity. The defect of ASS in the present case was not due to a reduced amount of ASSmRNA, but its structure was found to be abnormal; it was approximately 1.57 kb in length due to a defect of about 0.1 kb near the 3′ end of the coding region.
Precocious Puberty Caused by an hCG‐Producing Tumor of the Septum Pellucidum
Authors: Inamo Yasuji , Hiyoshi Kazuo , Hanawa Yoshio , Okuni Masahiko
Published: October 2007

A 6 year 9 month old boy with rapidly progressing precocious puberty was im‐munohistochemically and histologically diagnosed as having an hCG‐producing mixed tumor consisting of choriocarcinoma and teratoma in the septum pellucidum. His serum hCG was elevated, but the serum LH was low as determined by LH immunoradiometric assay (IRMA). He did not exhibit a characteristic endocrinological pattern, e.g., high basal levels of LH and failure to respond with high LH levels to the LH‐RH stimulation test using the conventional LH RIA method.
Chromosome Abnormalities and Prognosis in Childhood Acute Leukemia
Authors: Hayashi Yasuhide , Hanada Ryoji , Yamamoto Keiko
Published: October 2007

We report here on the leukemic cell karyotypes of 134 children with acute nonlymphocytic leukemia (ANLL) examined at Saitama Children's Medical Center (SCMC), and of 88 children with acute lymphoblastic leukemia (ALL) referred to SCMC. The patients were mainly treated according to the protocol of the Tokyo Children's Cancer Study Group. Of 106 ANLL cases with adequate banding, 18% were normal, 34% had miscellaneous clonal abnormalities, and 48% were classified into known cytogenetic subgroups: t(8;21) (n = 21), 11q23 abnormalities (n=14), ‐7/del(7q) (n = 6), inv (16)/del(16) (n = 5), and t(15;17) (n = 5). According to the FAB classification, M7 (21.7%) were more frequent than in previous reports because this study included a number of Down's Syndrome patients with M7 morphology. The present study confirmed the well‐known association of t(15; 17) with M3, t(8;21) with M2, 11q23 abnormalities with M4 and MS, and inv (16)/del(16) with M4. Patients with t(8;21) or inv (16)/del(16q) ANLL fared no better overall than the entire group. Of 51 ALL cases with adequate banding, 13.7% were normal, and 86.3% were classified into abnormal subgroups: translocation (n=14), hyperdiploidy (>50) (n=13), and miscellaneous abnormalities (n = 17). Cases with hyperdiploidy (>50) were restricted to a common phenotype and fared better overall than the entire group. Patients with translocation were found in all phenotypes, and had a poor prognosis. We concluded that childhood acute leukemia could be subgrouped according to karyotypic patterns, and that patients with translocations had a poor prognosis in ALL as well as ANLL.