Abstract:
:In seven patients with mucopolysaccharidoses (1 Hurler, 1 Hurler-Scheie, 4 Hunter, 1 Sly), cranial (1)H-magnetic resonance spectroscopy was performed to evaluate the accumulation of mucopolysaccharides and biochemical changes in the CNS in vivo before and after bone marrow transplantation (BMT). In two of seven patients, (1)H-magnetic resonance spectroscopy was performed before and after BMT. Nuclear magnetic resonance spectra of dermatan sulfate and chondroitin sulfate-C and magnetic resonance spectroscopy of chondroitin sulfate-C and urine from patients with mucopolysaccharidoses showed resonance higher than the chemical shift of myoinositol in the brain (3.7 ppm). The resonance was considered to contain signals from mucopolysaccharide molecules. The resonance was measured as presumptive mucopolysaccharides (pMPS). In white matter lesions detected by magnetic resonance imaging, pMPS/creatine ratios and choline/creatine ratios were consistently higher than control ratios. In white matter without lesions, choline/creatine ratios were higher than control ratios. Patients with higher developmental quotient or intelligence quotient tended to show higher N:-acetylaspartate/creatine ratios and lower pMPS/creatine ratios in basal ganglia. After BMT, the pMPS/creatine ratio in white matter lesions of patient 3, with Hunter syndrome, was slightly decreased, but in none of the patients was the ratio ever below the control ratios, even 7 y after BMT. In white matter without lesions, the pMPS/creatine ratio in patient 3 was decreased to the control ratios after BMT, but although the choline/creatine ratios were gradually decreased, they remained higher than the control ratio, 2 y after BMT. These results suggest that evaluation of pMPS, choline, and N:-acetylaspartate by (1)H-magnetic resonance spectroscopy is an important technique that may provide useful biochemical information in vivo on the neurologic process and the efficacy of BMT in patients with mucopolysaccharidoses.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Takahashi Y,Sukegawa K,Aoki M,Ito A,Suzuki K,Sakaguchi H,Watanabe M,Isogai K,Mizuno S,Hoshi H,Kuwata K,Tomatsu S,Kato S,Ito T,Kondo N,Orii Tdoi
10.1203/00006450-200103000-00008subject
Has Abstractpub_date
2001-03-01 00:00:00pages
349-55issue
3eissn
0031-3998issn
1530-0447journal_volume
49pub_type
杂志文章abstract::We examined the effect of inspiratory resistive loaded breathing (IRL) on the electromyographic (EMG) activity of the costal and crural diaphragm in nine anesthetized spontaneously breathing piglets (age 10-23 days, weight 2.8-4.4 kg). Bipolar wire electrodes were inserted into the anterior paratendinous costal diaphr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198701000-00007
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journal_title:Pediatric research
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doi:10.1203/00006450-199804000-00016
更新日期:1998-04-01 00:00:00
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journal_title:Pediatric research
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更新日期:1985-09-01 00:00:00
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journal_title:Pediatric research
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doi:10.1203/00006450-198604000-00006
更新日期:1986-04-01 00:00:00
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更新日期:2008-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:
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journal_title:Pediatric research
pub_type: 临床试验,杂志文章
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更新日期:1997-03-01 00:00:00
abstract::The age-related changes in fasting arterial levels of energy substrates and insulin were studied at birth and/or 6 wk in eleven baboon infants. In addition, the renal contribution to glucose release in the primate infant was estimated. Arterial blood glucose levels were similar in six fasted newborns and in nine faste...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198008000-00006
更新日期:1980-08-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199606000-00001
更新日期:1996-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.108
更新日期:2017-09-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199702000-00009
更新日期:1997-02-01 00:00:00
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journal_title:Pediatric research
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doi:10.1203/00006450-198202000-00010
更新日期:1982-02-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章,评审
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更新日期:2016-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1203/00006450-198804000-00003
更新日期:1988-04-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.243
更新日期:2014-03-01 00:00:00
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pub_type: 杂志文章
doi:10.1203/01.PDR.0000132750.97066.B9
更新日期:2004-08-01 00:00:00
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doi:10.1203/PDR.0b013e3181e5c3a4
更新日期:2010-08-01 00:00:00
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journal_title:Pediatric research
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更新日期:2005-02-01 00:00:00
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pub_type: 杂志文章
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更新日期:1997-06-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199904010-00007
更新日期:1999-04-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
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更新日期:2017-04-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
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更新日期:2003-02-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181d35034
更新日期:2010-05-01 00:00:00
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更新日期:2006-01-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198802000-00004
更新日期:1988-02-01 00:00:00
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journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1038/pr.2014.201
更新日期:2015-03-01 00:00:00
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journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198104000-00008
更新日期:1981-04-01 00:00:00