Acta paediatrica Japonica : Overseas edition最新文献_第8页

Tyrosinemia type I-like disease: a possible manifestation of 3-oxo-delta 4-steroid 5 beta-reductase deficiency. 酪氨酸血症i型样病:3-氧- δ 4-类固醇5 β -还原酶缺乏的可能表现

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01

A Kimura, F Endo, S Kagimoto, T Inoue, M Suzuki, T Kurosawa, M Tohma, T Fujisawa, H Kato

{"title":"Tyrosinemia type I-like disease: a possible manifestation of 3-oxo-delta 4-steroid 5 beta-reductase deficiency.","authors":"A Kimura, F Endo, S Kagimoto, T Inoue, M Suzuki, T Kurosawa, M Tohma, T Fujisawa, H Kato","doi":"","DOIUrl":"","url":null,"abstract":"Background: It has been suggested that quantitative analysis of urinary bile acids may help to distinguish primary 3-oxo-delta 4-steroid 5 beta-reductase deficiency from the excretion of 3-oxo-delta 4 bile acids that occurs as a result of liver damage.Methods: Urinary bile acids were quantitatively analyzed by gas chromatography-mass spectrometry in four Japanese patients with severe neonatal cholestasis associated with hypertyrosinemia without urinary succinylacetone (i.e. tyrosinemia type I-like disease). These four patients represented sporadic cases.Results: Large amounts of 3-oxo-delta 4 bile acids were detected, which comprised greater than 80% of the total urinary bile acids. Small amounts of allo-bile acids and primary bile acids were also detected, comprising less than 1% and 15% of the total urinary bile acids, respectively.Conclusions: It was suspected that these four patients had a primary 3-oxo-delta 4-steroid 5 beta-reductase deficiency. However, it is possible that some patients in this study may have had a secondary 3-oxo-delta 4-steroid 5 beta-reductase deficiency, caused by idiopathic neonatal cholestatic liver failure.","PeriodicalId":76975,"journal":{"name":"Acta paediatrica Japonica : Overseas edition","volume":"40 3","pages":"211-7"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20610889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Coronary risk factors in acute Kawasaki disease: correlation of serum immunoglobulin levels with coronary complications. 急性川崎病冠状动脉危险因素:血清免疫球蛋白水平与冠状动脉并发症的相关性

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01915.x

Y Sawaji, N Haneda, S Yamaguchi, Y Kajino, K Kishida, S Seto, N Konishi, K Waki, K Baba, K Arisawa, T Kamiya, C Mori

{"title":"Coronary risk factors in acute Kawasaki disease: correlation of serum immunoglobulin levels with coronary complications.","authors":"Y Sawaji, N Haneda, S Yamaguchi, Y Kajino, K Kishida, S Seto, N Konishi, K Waki, K Baba, K Arisawa, T Kamiya, C Mori","doi":"10.1111/j.1442-200x.1998.tb01915.x","DOIUrl":"https://doi.org/10.1111/j.1442-200x.1998.tb01915.x","url":null,"abstract":"Background: To determine the usefulness of the IgG z-score (age and sex-standardized serum IgG level) before intravenous gamma globulin therapy (IVGG) in predicting the occurrence or severity of coronary complications in Kawasaki disease (KD).Methods: A case-control study of clinical and laboratory findings with 88 children in the early stage of acute KD who received IVGG (100 or 200 mg/kg for 2-5 days) therapy. Of these, 20 cases had persistent coronary arterial lesions (small aneurysm, moderate aneurysm or large aneurysm persisting more than 1 month). The controls comprised 68 children with no coronary aneurysms or transient small aneurysm only observed within 1 month after the onset of KD. The association between serum levels of immunoglobulin G (IgG). IgM, IgA as well as other coronary risk factors previously reported and the occurrence of the coronary arterial lesions was evaluated using logistic regression analysis.Results: After adjustment for age, gender, total IVGG dose before the 9th illness day and other traditional coronary risk factors, the odds ratio for the persistent coronary aneurysm associated with lower serum IgG z-score (< -0.7485 vs > or = -0.7485), was 30.3 (95% confidence interval, 3.8-243.2). Furthermore, the serum IgG z-score was inversely correlated with the severity of the coronary arterial lesion.Conclusions: The IgG z-score before IVGG therapy in the early stage of KD provides useful information on the risk factors for persistent coronary aneurysm and is a novel, additional indicator for therapy to prevent the coronary complications in acute KD.","PeriodicalId":76975,"journal":{"name":"Acta paediatrica Japonica : Overseas edition","volume":"40 3","pages":"218-25"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1442-200x.1998.tb01915.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20610890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 24

Transient hypocalcemia with elevated serum parathormone in an infant of a hyperparathyroid mother. 甲状旁腺功能亢进母亲的婴儿短暂性低血钙伴血清甲状旁腺激素升高。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01931.x

E A Ismail, N al-Shammari, H Nadi

引用次数: 3

Changes in the duration of the urinary abnormality of purpura nephritis over the past 5 years. 紫癜性肾炎泌尿异常持续时间5年的变化。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01918.x

T Asano, T Nachi, K Maruyama, T Morita, M Murakami, M Yamamoto

引用次数: 1

Seasonal benzathine penicillin G prophylaxis for recurrent streptococcal pharyngitis in children. 季节性苄星青霉素G预防儿童复发性链球菌性咽炎。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01923.x

S Akşit, S Caglayan, G Dokucu

引用次数: 7

Successful pregnancy after coronary artery bypass grafting for Kawasaki disease. 川崎病冠状动脉旁路移植术后妊娠成功。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01927.x

H Hayakawa, T Katoh

引用次数: 11

Antineutrophil cytoplasmic antibody-positive crescentic glomerulonephritis associated with propylthiouracil therapy. 抗中性粒细胞胞浆抗体阳性的新月形肾小球肾炎与丙硫尿嘧啶治疗相关。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01930.x

M Fujieda, M Nagata, Y Akioka, M Hattori, H Kawaguchi, K Ito

引用次数: 13

Spectrophotometric determination of neutrophil cytochrome b558 of chronic granulomatous disease. 分光光度法测定慢性肉芽肿病中性粒细胞细胞色素b558。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01913.x

C Kiryu, M Kaneda, T Shiraishi, M Tsuda, I Inana, T Sakiyama, T Fujinaga, A Nishida, K Kakinuma

{"title":"Spectrophotometric determination of neutrophil cytochrome b558 of chronic granulomatous disease.","authors":"C Kiryu, M Kaneda, T Shiraishi, M Tsuda, I Inana, T Sakiyama, T Fujinaga, A Nishida, K Kakinuma","doi":"10.1111/j.1442-200x.1998.tb01913.x","DOIUrl":"https://doi.org/10.1111/j.1442-200x.1998.tb01913.x","url":null,"abstract":"Background: Chronic granulomatous disease (CGD) is an inherited disease characterized clinically by severe recurrent bacterial infections from infancy. This disease is a disorder of the formation of superoxide (O2-) by the neutrophil NADPH oxidase system, mostly due to defects in cytochrome b558 (cyt b558), which is one of the oxidase components. Diagnosis of CGD has been performed by the assay of the O2- forming activity, immunological determination of defects in the oxidase components, and or spectrophotometry of cyt b558. However, spectrophotometric analysis of the b-type heme is difficult with small amounts of blood from infant CGD patients, as the limited amounts of neutrophils are contaminated with a relatively high ratio of hemoglobin (Hb) that interferes with the heme spectrum of cyt b558. This report presents an accurate method for the spectrophotometric analysis of cyt b558 in a small amount of CGD neutrophils that were treated with CO gas in a safe procedure instead of the previously reported CO-bubbling method.Methods and results: The difference of the reduced minus oxidized cyt b558 spectrum was measured under no interference from oxy Hb at the alpha and beta bands and differentiated as d[delta A]/d lambda (lambda = wavelength) to obtain further evidence for the defects of the cyt b558 heme spectrum. The interference from CO-insensitive met Hb was eliminated by subtracting the absorption peak at the Soret (gamma) band of the contaminating met Hb, which was estimated from the CO-treated and untreated spectra of the same, hemolyzed sample.Conclusions: This spectrophotometric method is feasible for the determination of abnormality and heme content of cyt b558 with a small amount of CGD neutrophils in 10-20 mL of blood even in the presence of contaminating Hb.","PeriodicalId":76975,"journal":{"name":"Acta paediatrica Japonica : Overseas edition","volume":"40 3","pages":"204-10"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1442-200x.1998.tb01913.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20610888","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Using polymerase chain reaction for rapid sex-determination of a case of sirenomelia. 应用聚合酶链反应快速测定1例鼻疽病例的性别。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01929.x

Y Jin, A Endo, M Shimada, M Minato, M Takada, S Takahashi, K Harada, S Hayakawa, K Sato

引用次数: 2

National survey of periventricular leukomalacia in Japan. 日本脑室周围白质软化症的全国调查。

Acta paediatrica Japonica : Overseas edition Pub Date : 1998-06-01 DOI: 10.1111/j.1442-200x.1998.tb01919.x

S Fujimoto, H Togari, S Takashima, M Funato, H Yoshioka, S Ibara, M Tatsuno

{"title":"National survey of periventricular leukomalacia in Japan.","authors":"S Fujimoto, H Togari, S Takashima, M Funato, H Yoshioka, S Ibara, M Tatsuno","doi":"10.1111/j.1442-200x.1998.tb01919.x","DOIUrl":"https://doi.org/10.1111/j.1442-200x.1998.tb01919.x","url":null,"abstract":"Background: Clarification of the present status of periventricular leukomalacia (PVL) in Japan.Methods: Questionnaires were mailed to the leading neonatal intensive care units (NICU) and rehabilitation centers for children.Results: The incidence of PVL in the group of surviving preterm infants of gestational ages less than 33 weeks was 4.8-4.9% on ultrasound and 7.7-7.9% on magnetic resonance imaging and/or computed tomography. The incidence of PVL did not decrease between 1990 and 1994. The incidence of PVL in the NICU varied from 0 to 47.1%. Infants from multiple pregnancies had a higher incidence (9.1%) of PVL than those from single pregnancies (6.2%). The proportion of PVL in all patients in rehabilitation centers with cerebral palsy (CP) has increased recently.Conclusions: It was roughly calculated that about 750 cases of CP with PVL occurred annually in Japan, thus accounting for about one-third of the total number of cases of CP.","PeriodicalId":76975,"journal":{"name":"Acta paediatrica Japonica : Overseas edition","volume":"40 3","pages":"239-43"},"PeriodicalIF":0.0,"publicationDate":"1998-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1111/j.1442-200x.1998.tb01919.x","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"20611424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 20