Abstract:
:We addressed growth of biliary atresia (BA) patients living with native livers between ages 0-6 and effects of post-surgical corticosteroid treatment on growth. Growth charts of 28 BA patients born in Finland between 1987 and 2017 were retrospectively evaluated. Dosage and length of corticosteroid treatment and hydrocortisone substitution were reviewed. At birth, BA patients were shorter (median height - 0.6 (interquartile range (IQR) - 1.3 to - 0.1) SDS, n = 28, P < 0.001) than general population. Height remained stable during early childhood (median height - 0.6 (IQR - 1.4 to 0.1) SDS for girls and - 0.4 (IQR - 1.6 to 0.2) SDS for boys at 6 years of age). Patients were of normal height adjusted weight at 6 years with a median age and sex-adjusted body mass index (ISO-BMI) of 20.9 (IQR 19.3 to 25.0) for girls and 22.1 (IQR 20.7 to 25.6) for boys. Higher (≥ 50 mg/kg) cumulative post-portoenterostomy prednisolone dosage resulted in 0.18 SDS lower height per treatment week (β - 0.18, SE 0.04, P < 0.001) compared to lower dosage (< 50 mg/kg).Conclusion: BA patients grow normally during early childhood. As high postoperative corticosteroid dosage has a short-term negative effect on height, very high dosages should be avoided. What Is Known: • Growth of biliary atresia patients has mostly been shown to be within normal limits • Corticosteroids may decrease growth rate What Is New: • Biliary atresia patients surviving with their native livers are shorter than general population and their mid-parental target height at birth • A high (> 50 mg/kg) cumulative prednisolone dosage has a negative transitory impact on height gain after portoenterostomy.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Ruuska SM,Lääperi MT,Hukkinen M,Jalanko H,Kolho KL,Pakarinen MPdoi
10.1007/s00431-018-3302-zsubject
Has Abstractpub_date
2019-03-01 00:00:00pages
341-349issue
3eissn
0340-6199issn
1432-1076pii
10.1007/s00431-018-3302-zjournal_volume
178pub_type
杂志文章abstract::A 4-year-old boy is described with abdominal pain, emesis, weight loss, hypoproteinemia and edema. The diagnosis of Menetrier disease was made based on radiographic studies, gastroscopy and gastric biopsy. There was little response to medical treatment and enteral feedings were poorly tolerated for many weeks. Althoug...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441749
更新日期:1986-02-01 00:00:00
abstract:UNLABELLED:Transient hypothyroxinaemia with normal thyroid stimulating hormone (TSH) levels is a well-known condition in preterm neonates and is generally assumed to be a harmless epiphenomenon of prematurity. This assumption is, however, based on studies that included very few neonates with a gestational age (GA) belo...
journal_title:European journal of pediatrics
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doi:10.1007/BF01953940
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00446056
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02074831
更新日期:1995-07-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
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abstract:UNLABELLED:Fibronectin (a glycoprotein which modulates inflammation) may decrease mortality in systemic infection. Children with meningococcal disease (MCD) may have low fibronectin levels. We aimed to compare plasma fibronectin levels in children with MCD and controls, correlate fibronectin levels with interleukin-6 (...
journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1867-5
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-3014-9
更新日期:2017-12-01 00:00:00
abstract::Complete masculinisation of the external genitalia (type V of Prader classification) is reported in two siblings with normal internal female genitalia, normal ovaries and a 46,XX chromosome constitution in different cell lines without any detectable source of exposure to androgen during pregnancy. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445596
更新日期:1984-06-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957685
更新日期:1990-05-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02073374
更新日期:1993-10-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01846034
更新日期:1980-08-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1792-7
更新日期:2012-10-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-012-1710-z
更新日期:2013-02-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00496087
更新日期:1980-05-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00014231
更新日期:1996-07-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050986
更新日期:1998-12-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014313
更新日期:1999-12-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00647276
更新日期:1987-01-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02073895
更新日期:1992-01-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究,评审
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更新日期:2002-01-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-003-1384-7
更新日期:2004-03-01 00:00:00
abstract::Triple A syndrome (TAS) or Allgrove syndrome (OMIM #231550) is a rare autosomal recessive disorder characterised by adrenocorticotropic hormone-resistant adrenal insufficiency, alacrima, achalasia, and neurological and dermatological abnormalities. Mutations in the AAAS gene on chromosome 12q13 encoding the nuclear po...
journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:2018-03-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-014-2470-8
更新日期:2015-06-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章,多中心研究
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更新日期:2006-12-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00496426
更新日期:1988-05-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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journal_title:European journal of pediatrics
pub_type: 杂志文章
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更新日期:2014-01-01 00:00:00
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journal_title:European journal of pediatrics
pub_type: 信件
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更新日期:2002-03-01 00:00:00