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SHINICHI KOBAYASHI KAORU HIGUCHI HISASHI TAMAKI YASUYUKI WADA NORIYUKI WADA MASAKATSU KUBO YUICHI KOIKE MASATO NAGATA OSAMU TATSUZAWA SATOSHI FUJIKAWA 《Pediatrics international》1997,39(2):257-262
Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM , Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM. 相似文献
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H. TSUKAHARA M. HIRAOKA C. HORI S. TSUCHIDA H. UCHIDA K. FUJISAWA Y. KONISH M. SUDO 《Journal of paediatrics and child health》1996,32(4):330-332
Objective : To clarify postnatal changes in urinary uric acid (UA) excretion in normal term infants and to examine the effects of prematurity or illness on the UA excretion.
Methodology : Measurements of urinary UA were performed in term and premature infants at the ages of 1 and 7 days and at 1 and 4 months, as well as at 7 months in term infants.
Results : Urinary UA levels were lowest on day 7 in term infants. The levels were highest on day 1 in premature infants and remained significantly higher compared to term babies during the first month of life. Respiratory failure requiring ventilation and oxygen supply resulted in further significant elevation of urinary UA in premature infants.
Conclusions : With the reference values obtained in the study reported here, urinary UA can now be used for the diagnosis and monitoring of inherited disorders of purine metabolism and for the assessment of oxygen radical insult to sick infants. 相似文献
Methodology : Measurements of urinary UA were performed in term and premature infants at the ages of 1 and 7 days and at 1 and 4 months, as well as at 7 months in term infants.
Results : Urinary UA levels were lowest on day 7 in term infants. The levels were highest on day 1 in premature infants and remained significantly higher compared to term babies during the first month of life. Respiratory failure requiring ventilation and oxygen supply resulted in further significant elevation of urinary UA in premature infants.
Conclusions : With the reference values obtained in the study reported here, urinary UA can now be used for the diagnosis and monitoring of inherited disorders of purine metabolism and for the assessment of oxygen radical insult to sick infants. 相似文献
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AKIO NAKAI YOSUKE SHIGEMATSU YOSHIHARU KIKAWA MASAKATSU SUDO 《Pediatrics international》1994,36(4):408-411
A 7 years 3 months old Japanese boy with familial thyroxine binding globulin (TBG) excess associated with growth hormone (GH) deficiency is reported. The patients height was 106.4 cm (- 2.86 s.d.) and his bone age was 5 years and 3 months. He had no goiter and his developmental milestones were normal. The serum thyroid stimulating hormone (TSH) was 2.8 μU/mL, triiodothyronine (T3) 3.1 ng/mL, thyroxine (T4) 23.4 μg/dL and free T4 1.8 ng/dL. The serum TBG level was beyond 80.0 μg/mL, with normal TSH response to the thyrotropin-releasing hormone (TRH) test. Familial study revealed that his grandmother, mother, uncle, younger sister and younger brother had high TBG and T3 levels, thus an X-linked co-dominant transmission was suggested. The peak GH responses to insulin and clonidine hydrochloride were 5.8 and 8.2 ng/mL, respectively. The mean nocturnal GH concentration was 2.5 ng/mL. His growth velocity increased from 4.8 to 8.4 cm/year and his serum TBG levels decreased gradually after human growth hormone (hGH) treatment. 相似文献
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HIROKAZU TSUKAHARA TOSHIO IMURA SHINYA TSUCHIDA MITSUKO NUNOSE CHIKAHIDE HORI MASAHIRO HIRAOKA FUMITAKE GEJYO MASAKATSU SUDO 《Pediatrics international》1996,38(6):614-618
The purpose of the present study was to examine renal functional changes caused by chronic blockade of nitric oxide (NO) synthesis in young rats. Two types of NO synthase inhibitor were used: NG-nitro-L-arginine methyl ester (L-NAME) as a non-selective inhibitor and aminoguanidine (AG) as a selective inhibitor of the inducible isoform. Oral administration of L-NAME (20–80 mg/dL of drinking water), not AG (400 mg/dL), for 4 weeks induced systemic hypertension in the treated rats. Both inhibitors caused a significant reduction in urinary excretion of NO2?/NO3?. Rats treated with L-NAME developed proteinuria and tubular enzymuria (high excretion of N-acetyl-β-D-glucosaminidase) in a dose-dependent fashion, with normal serum levels of creatinine, albumin and cholesterol. Chronic AG administration did not alter the urinary levels of protein and N-acetyl-β-D-glucosaminidase or serum laboratory values. Overall, these observations highlight the importance of the continuous generation of NO by the constitutive isoform in the control of vascular tone and the maintenance of renal glomerular and tubular function. Oral administration of L-NAME may serve as a model of chronic NO-deficient hypertension with renal injury in young rats. 相似文献
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AKIHIRO SUDO YOSHIO OGIHARA ITSUO KUSANO TOKUHIRO UEMURA 《Journal of Medical Imaging and Radiation Oncology》1990,34(2):181-184
A case of melorheostosis in a 57-year-old Japanese woman is presented. This case is noteworthy because of its unusual radiographic features and local findings and its unusually long follow-up period of more than 29 years. Melorheostosis progresses very slowly over a long period of time, and can show bizarre features, simulating malignant tumor. 相似文献
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H TSUKAHARA S HARUKI M HIRAOKA C HORI M SUDO 《Journal of paediatrics and child health》1997,33(3):253-255
Objective: To investigate long-term changes of serum cholesterol levels in children with frequently relapsing steroid-responsive nephrotic syndrome (NS).
Methodology: Serum cholesterol values just before and during or immediately after 'relapse' were reviewed and the incidence of hypercholesterolaemia (≥200 mg/dL) was determined in eight patients (M:F, 6:2).
Results: The patients with frequently relapsing NS usually showed hypercholesterolaemia (mean incidence, 81%) just before 'relapse' during clinical remission, as well as in relapse (mean incidence, 96%). A high incidence of steroid therapy was also found in each case (mean, 89%) just before relapse.
Conclusions: Our results demonstrate that children with frequently relapsing NS have prolonged periods of hypercholesterolaemia, even during clinical remission. It is suggested that serum lipid profiles be monitored carefully in such patients. 相似文献
Methodology: Serum cholesterol values just before and during or immediately after 'relapse' were reviewed and the incidence of hypercholesterolaemia (≥200 mg/dL) was determined in eight patients (M:F, 6:2).
Results: The patients with frequently relapsing NS usually showed hypercholesterolaemia (mean incidence, 81%) just before 'relapse' during clinical remission, as well as in relapse (mean incidence, 96%). A high incidence of steroid therapy was also found in each case (mean, 89%) just before relapse.
Conclusions: Our results demonstrate that children with frequently relapsing NS have prolonged periods of hypercholesterolaemia, even during clinical remission. It is suggested that serum lipid profiles be monitored carefully in such patients. 相似文献
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SUGA MITSUO; SUDO MINORU; KOIKE HIROSHI; ODANI SHUNICHI; YACHI AKIRA; WADA TAKEO; CHISAKA NORIYASU; SATO YASUNORI; URASAWA KIICHI 《Japanese journal of clinical oncology》1979,9(1):131-137
An autopsy case of a nonfunctioning adrenal cortical carcinomais presented and the features of 16 cases with this tumor reportedbetween 1953 and 1977 in Japan are reviewed. This tumor predominantly afflicted males and the average ageof admission was 46.5 years. A palpable mass and abdominal painwere common symptoms. The average levels of 17-KS and 17-OHCSin the urine were 8.3 mg/24 hr and 4.6 mg/24 hr, respectively.The most common sites of distant metastases were the liver andlung. It was also noted that the only available mode of treatmentwas surgery. 相似文献
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